extracellular matrix proteins

Summary

Summary: Macromolecular organic compounds that contain carbon, hydrogen, oxygen, nitrogen, and usually, sulfur. These macromolecules (proteins) form an intricate meshwork in which cells are embedded to construct tissues. Variations in the relative types of macromolecules and their organization determine the type of extracellular matrix, each adapted to the functional requirements of the tissue. The two main classes of macromolecules that form the extracellular matrix are: glycosaminoglycans, usually linked to proteins (proteoglycans), and fibrous proteins (e.g., COLLAGEN; ELASTIN; FIBRONECTINS; and LAMININ).

Top Publications

  1. pmc The extracellular matrix: not just pretty fibrils
    Richard O Hynes
    Howard Hughes Medical Institute, David H Koch Institute for Integrative Cancer Research, Massachusetts Institute of Technology, Cambridge, MA 02139, USA
    Science 326:1216-9. 2009
  2. ncbi Spontaneous calcification of arteries and cartilage in mice lacking matrix GLA protein
    G Luo
    Department of Molecular Genetics, The University of Texas M D Anderson Cancer Center, Houston 77030, USA
    Nature 386:78-81. 1997
  3. pmc Genetic fate mapping reveals that the caudal ganglionic eminence produces a large and diverse population of superficial cortical interneurons
    Goichi Miyoshi
    Smilow Neuroscience Program and the Department of Cell Biology, New York University School of Medicine, New York, New York 10016, USA
    J Neurosci 30:1582-94. 2010
  4. pmc MicroRNA 29c is down-regulated in nasopharyngeal carcinomas, up-regulating mRNAs encoding extracellular matrix proteins
    Srikumar Sengupta
    Institute for Molecular Virology, Howard Hughes Medical Institute, McArdle Laboratory for Cancer Research, Departments of Statistics and Biostatistics and Medical Informatics, University of Wisconsin, Madison, WI 53076, USA
    Proc Natl Acad Sci U S A 105:5874-8. 2008
  5. pmc Loss of DMP1 causes rickets and osteomalacia and identifies a role for osteocytes in mineral metabolism
    Jian Q Feng
    Oral Biology, University of Missouri Kansas City, Kansas City, Missouri 64108, USA
    Nat Genet 38:1310-5. 2006
  6. ncbi Cell-surface and mitotic-spindle RHAMM: moonlighting or dual oncogenic functions?
    Christopher Alan Maxwell
    Translational Research Laboratory, Catalan Institute of Oncology, IDIBELL, L Hospitalet, Barcelona, Spain
    J Cell Sci 121:925-32. 2008
  7. ncbi Recombinant vitronectin is a functionally defined substrate that supports human embryonic stem cell self-renewal via alphavbeta5 integrin
    Stefan R Braam
    Leiden University Medical Centre, Postal Zone S 1 P, P O Box 9600, 2300 RC Leiden, The Netherlands
    Stem Cells 26:2257-65. 2008
  8. pmc Targeted disruption of decorin leads to abnormal collagen fibril morphology and skin fragility
    K G Danielson
    Department of Pathology, Anatomy, and Cell Biology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA
    J Cell Biol 136:729-43. 1997
  9. ncbi A protein related to extracellular matrix proteins deleted in the mouse mutant reeler
    G D'ARCANGELO
    Roche Institute of Molecular Biology, Hoffman La Roche, Nutley, New Jersey 07110, USA
    Nature 374:719-23. 1995
  10. ncbi Mutation of a gene encoding a protein with extracellular matrix motifs in Usher syndrome type IIa
    J D Eudy
    Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE 68198, USA
    Science 280:1753-7. 1998

Research Grants

  1. INSULIN RECEPTORS AND THE GLUCOSE TRANSPORT SYSTEM
    JERROLD MICHAEL OLEFSKY; Fiscal Year: 2013
  2. MECHANISMS OF RADIORESISTANCE AND CELL CYCLE PROGRESSION
    Howard B Lieberman; Fiscal Year: 2013
  3. Metabolic regulation of diabetic nephropathy
    Karen Block; Fiscal Year: 2013
  4. IGFBP-5 and Hsp70 in Extracellular Matrix Homeostasis and Fibrosis
    Kristen L Veraldi; Fiscal Year: 2013
  5. Mechanisms of Airway Smooth Muscle Hypertrophy
    Marc B Hershenson; Fiscal Year: 2013
  6. Genetic Analysis of FAK Activity
    David D Schlaepfer; Fiscal Year: 2012
  7. Role of DDR2 in OA-like Pathogenesis in Osteochondrodysplasias
    Yefu Li; Fiscal Year: 2010
  8. Role of Biophysical Cues in the Pathogenesis of Glaucoma
    Paul Russell; Fiscal Year: 2013
  9. Harness Germinal Matrix Hemorrhage
    John H Zhang; Fiscal Year: 2013
  10. PTH-RELATED PEPTIDE AND VIT D IN PROSTATE CANCER GROWTH
    Miriam Falzon; Fiscal Year: 2009

Detail Information

Publications329 found, 100 shown here

  1. pmc The extracellular matrix: not just pretty fibrils
    Richard O Hynes
    Howard Hughes Medical Institute, David H Koch Institute for Integrative Cancer Research, Massachusetts Institute of Technology, Cambridge, MA 02139, USA
    Science 326:1216-9. 2009
    ..As organized, solid-phase ligands, ECM proteins can integrate complex, multivalent signals to cells in a spatially patterned and regulated fashion. These properties need to be incorporated into considerations of the functions of the ECM...
  2. ncbi Spontaneous calcification of arteries and cartilage in mice lacking matrix GLA protein
    G Luo
    Department of Molecular Genetics, The University of Texas M D Anderson Cancer Center, Houston 77030, USA
    Nature 386:78-81. 1997
    ..These results indicate that ECM calcification must be actively inhibited in soft tissues. To our knowledge, Mgp is the first inhibitor of calcification of arteries and cartilage to be characterized in vivo...
  3. pmc Genetic fate mapping reveals that the caudal ganglionic eminence produces a large and diverse population of superficial cortical interneurons
    Goichi Miyoshi
    Smilow Neuroscience Program and the Department of Cell Biology, New York University School of Medicine, New York, New York 10016, USA
    J Neurosci 30:1582-94. 2010
    ..Thus, in conjunction with previous studies, we have now determined the spatial and temporal origins of the vast majority of cortical interneuron subtypes...
  4. pmc MicroRNA 29c is down-regulated in nasopharyngeal carcinomas, up-regulating mRNAs encoding extracellular matrix proteins
    Srikumar Sengupta
    Institute for Molecular Virology, Howard Hughes Medical Institute, McArdle Laboratory for Cancer Research, Departments of Statistics and Biostatistics and Medical Informatics, University of Wisconsin, Madison, WI 53076, USA
    Proc Natl Acad Sci U S A 105:5874-8. 2008
    ..Most of the mir-29c-targeted genes identified encode extracellular matrix proteins, including multiple collagens and laminin gamma1, that are associated with tumor cell invasiveness and ..
  5. pmc Loss of DMP1 causes rickets and osteomalacia and identifies a role for osteocytes in mineral metabolism
    Jian Q Feng
    Oral Biology, University of Missouri Kansas City, Kansas City, Missouri 64108, USA
    Nat Genet 38:1310-5. 2006
    ..Our findings suggest a bone-renal axis that is central to guiding proper mineral metabolism...
  6. ncbi Cell-surface and mitotic-spindle RHAMM: moonlighting or dual oncogenic functions?
    Christopher Alan Maxwell
    Translational Research Laboratory, Catalan Institute of Oncology, IDIBELL, L Hospitalet, Barcelona, Spain
    J Cell Sci 121:925-32. 2008
    ..The unconventional export of proteins such as RHAMM is a novel process that modifies the roles of tumor suppressors and promoters, such as BRCA1 and CD44, and might provide new targets for therapeutic intervention...
  7. ncbi Recombinant vitronectin is a functionally defined substrate that supports human embryonic stem cell self-renewal via alphavbeta5 integrin
    Stefan R Braam
    Leiden University Medical Centre, Postal Zone S 1 P, P O Box 9600, 2300 RC Leiden, The Netherlands
    Stem Cells 26:2257-65. 2008
    ..Recombinant vitronectin was the only defined functional alternative to Matrigel, supporting sustained self-renewal and pluripotency in three independent hESC lines...
  8. pmc Targeted disruption of decorin leads to abnormal collagen fibril morphology and skin fragility
    K G Danielson
    Department of Pathology, Anatomy, and Cell Biology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA
    J Cell Biol 136:729-43. 1997
    ..These findings demonstrate a fundamental role for decorin in regulating collagen fiber formation in vivo...
  9. ncbi A protein related to extracellular matrix proteins deleted in the mouse mutant reeler
    G D'ARCANGELO
    Roche Institute of Molecular Biology, Hoffman La Roche, Nutley, New Jersey 07110, USA
    Nature 374:719-23. 1995
    ..The encoded protein resembles extracellular matrix proteins involved in cell adhesion...
  10. ncbi Mutation of a gene encoding a protein with extracellular matrix motifs in Usher syndrome type IIa
    J D Eudy
    Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE 68198, USA
    Science 280:1753-7. 1998
    ....
  11. ncbi Reelin, lipoprotein receptors and synaptic plasticity
    Joachim Herz
    Department of Molecular Genetics, University of Texas Southwestern Medical Center, Dallas, Texus 75390, USA
    Nat Rev Neurosci 7:850-9. 2006
    ..Here, we review the molecular mechanisms through which APOE, cholesterol, reelin and APOE receptors control synaptic functions that are essential for cognition, learning, memory, behaviour and neuronal survival...
  12. pmc Interaction of the small interstitial proteoglycans biglycan, decorin and fibromodulin with transforming growth factor beta
    A Hildebrand
    Cancer Research Center, La Jolla Cancer Research Foundation, CA 92037
    Biochem J 302:527-34. 1994
    ..They may regulate TGF-beta activities by sequestering TGF-beta into extracellular matrix...
  13. pmc Reelin promoter hypermethylation in schizophrenia
    Dennis R Grayson
    Psychiatric Institute, Department of Psychiatry, College of Medicine, University of Illinois, 1601 West Taylor Street, Chicago, IL 60612, USA
    Proc Natl Acad Sci U S A 102:9341-6. 2005
    ..Collectively, the data show that this promoter region has positive and negative properties and that the function of this complex cis element relates to its methylation status...
  14. pmc VEGF-A and Tenascin-C produced by S100A4+ stromal cells are important for metastatic colonization
    Joyce T O'Connell
    Division of Matrix Biology, Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 108:16002-7. 2011
    ..Our study demonstrates a crucial role for local S100A4(+) fibroblasts in providing the permissive "soil" for metastatic colonization, a challenging step in the metastatic cascade...
  15. pmc A novel form of chondrocyte stress is triggered by a COMP mutation causing pseudoachondroplasia
    Farhana Suleman
    Wellcome Trust Centre for Cell Matrix Research, Faculty of Life Sciences, The University of Manchester, Manchester, United Kingdom
    Hum Mutat 33:218-31. 2012
    ..Overall, these data suggest that a novel form of chondrocyte stress triggered by the expression of mutant COMP is central to the pathogenesis of PSACH...
  16. pmc Cartilage oligomeric matrix protein/thrombospondin 5 supports chondrocyte attachment through interaction with integrins
    Faye Hui Chen
    Department of Orthopaedic Surgery, Columbia University, New York, New York 10032, USA
    J Biol Chem 280:32655-61. 2005
    ..Through these interactions, COMP/TSP5 may be able to regulate cellular activities and respond to environment in the surrounding cartilage matrix...
  17. ncbi Medial localization of mineralization-regulating proteins in association with Mönckeberg's sclerosis: evidence for smooth muscle cell-mediated vascular calcification
    C M Shanahan
    Department of Medicine, Addenbrooke s Hospital, Cambridge, UK
    Circulation 100:2168-76. 1999
    ..Therefore, we examined gene expression in vessels with MS to determine whether there was evidence for a regulated calcification process in the vessel media...
  18. ncbi A new secreted protein that binds to Wnt proteins and inhibits their activities
    J C Hsieh
    Department of Molecular Biology and Genetics, Howard Hughes Medical Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Nature 398:431-6. 1999
    ....
  19. ncbi Reelin and brain development
    Fadel Tissir
    Developmental Genetics Unit, Universite Catholique de Louvain, UCL 7382, 73 Avenue E Mounier, B1200 Brussels, Belgium
    Nat Rev Neurosci 4:496-505. 2003
  20. pmc Extracellular matrix molecules: potential targets in pharmacotherapy
    Hannu Jarvelainen
    Department of Medicine, Turku University Hospital, Kiinamyllynkatu 4 8, FI 20520 Turku, Finland
    Pharmacol Rev 61:198-223. 2009
    ..In the future, agents and compounds targeting directly the ECM will significantly advance the treatment of various human diseases, even those for which efficient therapies are not yet available...
  21. pmc Small integrin-binding ligand N-linked glycoproteins (SIBLINGs): multifunctional proteins in cancer
    Akeila Bellahcene
    Metastasis Research Laboratory, University of Liege, Tour de Pathologie, 1, Bat B23, Sart Tilman via 4000 Liege, Belgium
    Nat Rev Cancer 8:212-26. 2008
    ....
  22. pmc Role of extracellular matrix proteins and their receptors in the development of the vertebrate neuromuscular junction
    Neha Singhal
    Center for Gene Therapy, The Research Institute at Nationwide Children s Hospital, Department of Pediatrics, Ohio State University College of Medicine, Columbus, Ohio 43205, USA
    Dev Neurobiol 71:982-1005. 2011
    ..Synaptic ECM proteins and their receptors are involved in almost all aspects of synaptic development, including synaptic initiation, topography, ultrastructure, maturation, stability, and transmission...
  23. ncbi Molecular basis of human Usher syndrome: deciphering the meshes of the Usher protein network provides insights into the pathomechanisms of the Usher disease
    Jan Reiners
    Institute of Zoology, Department of Cell and Matrix Biology, Johannes Gutenberg University of Mainz, Müllerweg 6, D 55099 Mainz, Germany
    Exp Eye Res 83:97-119. 2006
    ....
  24. ncbi Reelin is a ligand for lipoprotein receptors
    G D'ARCANGELO
    Department of Developmental Neurobiology, St Jude Children s Research Hospital, Memphis, Tennessee 38105, USA
    Neuron 24:471-9. 1999
    ..In dissociated neurons, apoE reduces the level of Reelin-induced tyrosine phosphorylation of Dab1. These data suggest that Reelin directs neuronal migration by binding to VLDLR and ApoER2...
  25. pmc How matrix metalloproteinases regulate cell behavior
    M D Sternlicht
    Department of Anatomy, University of California, San Francisco, California 94143 0452, USA
    Annu Rev Cell Dev Biol 17:463-516. 2001
    ..proteins, cell surface receptors, cell-cell adhesion molecules, and virtually all structural extracellular matrix proteins. Thus MMPs are able to regulate many biologic processes and are closely regulated themselves...
  26. ncbi Aberrant expression of collagen triple helix repeat containing 1 in human solid cancers
    Liren Tang
    Department of Dermatology and Skin Science, University of British Columbia, Vancouver, British Columbia, Canada
    Clin Cancer Res 12:3716-22. 2006
    ..However, the full significance of CTHRC1 to human carcinogenesis remains unclear. This study is to further characterize the clinical and functional relevance of CTHRC1 in melanoma and other human solid cancers...
  27. ncbi Regulation of endoplasmic reticulum stress response by a BBF2H7-mediated Sec23a pathway is essential for chondrogenesis
    Atsushi Saito
    Division of Molecular and Cellular Biology, Department of Anatomy, Faculty of Medicine, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889 1692, Japan
    Nat Cell Biol 11:1197-204. 2009
    ..zone and a decrease in the size of the hypertrophic zone, resulting in a significant reduction of extracellular matrix proteins. Interestingly, proliferating chondrocytes showed abnormally expanded ER, containing aggregated type ..
  28. pmc The hyaluronan receptors CD44 and Rhamm (CD168) form complexes with ERK1,2 that sustain high basal motility in breast cancer cells
    Sara R Hamilton
    London Regional Cancer Program, London Health Sciences Centre, and the University of Western Ontario, London, and Department of Cardiovascular Research, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada
    J Biol Chem 282:16667-80. 2007
    ..Therefore, an effect of CD44 on tumor cell motility may depend in part on its ability to partner with additional proteins, such as cell surface Rhamm...
  29. pmc The molecular basis of corneal transparency
    John R Hassell
    Department of Molecular Medicine, College of Medicine, University of South Florida, Tampa, FL 33612 4799, USA
    Exp Eye Res 91:326-35. 2010
    ....
  30. pmc Pseudoachondroplasia and multiple epiphyseal dysplasia: a 7-year comprehensive analysis of the known disease genes identify novel and recurrent mutations and provides an accurate assessment of their relative contribution
    Gail C Jackson
    Wellcome Trust Centre for Cell Matrix Research, University of Manchester, Manchester, United Kingdom
    Hum Mutat 33:144-57. 2012
    ..Furthermore, this study provides the first indication of the relative contribution of each gene and confirms that they account for the majority of PSACH and MED...
  31. ncbi EFEMP1 expression promotes angiogenesis and accelerates the growth of cervical cancer in vivo
    En Lin Song
    Biomedical Engineering Center, Department of Anatomy, the Zhongshan School of Medicine, Sun Yat Sen University, Guangzhou, Guangdong 510080, China
    Gynecol Oncol 121:174-80. 2011
    ..The study was to investigate the role of EFEMP1 in angiogenesis and growth of cervical carcinoma in vivo...
  32. ncbi TGF-beta signaling and the fibrotic response
    Andrew Leask
    Centre for Rheumatology, Department of Medicine, Royal Free and University College Medical School, Rowland Hill St, London, UK NW3 2PF
    FASEB J 18:816-27. 2004
    ....
  33. ncbi A novel Usher protein network at the periciliary reloading point between molecular transport machineries in vertebrate photoreceptor cells
    Tina Maerker
    Department of Cell and Matrix Biology, Institute of Zoology, Johannes Gutenberg University of Mainz, 55099 Mainz, Germany
    Hum Mol Genet 17:71-86. 2008
    ..Since this structural specialization in amphibian photoreceptor cells defines a specialized membrane domain for docking and fusion of transport vesicles, we suggest a prominent role of the USH proteins in cargo shipment...
  34. pmc The extracellular matrix protein TGFBI induces microtubule stabilization and sensitizes ovarian cancers to paclitaxel
    Ahmed Ashour Ahmed
    Functional Genomics of Drug Resistance Laboratory, Cancer Research UK Cambridge Research Institute, Li Ka Shing Centre, Robinson Way, Cambridge CB2 0RE, UK
    Cancer Cell 12:514-27. 2007
    ..These data show that ECM can mediate taxane sensitivity by modulating microtubule stability...
  35. pmc Reelin regulates cadherin function via Dab1/Rap1 to control neuronal migration and lamination in the neocortex
    Santos J Franco
    Dorris Neuroscience Center and Department of Cell Biology, The Scripps Research Institute, La Jolla, CA 92037, USA
    Neuron 69:482-97. 2011
    ....
  36. ncbi Osteomalacia in hyp mice is associated with abnormal phex expression and with altered bone matrix protein expression and deposition
    D Miao
    Department of Medicine, McGill University, Montreal, Quebec, Canada H3A 1A1
    Endocrinology 142:926-39. 2001
    ..These results suggest that alterations in the Phex gene may control bone matrix mineralization indirectly by regulating the synthesis and deposition of bone matrix proteins...
  37. pmc The basement membrane of hair follicle stem cells is a muscle cell niche
    Hironobu Fujiwara
    Cancer Research UK Cambridge Research Institute, Li Ka Shing Centre, UK
    Cell 144:577-89. 2011
    ..Thus, bulge stem cells, via nephronectin expression, create a smooth muscle cell niche and act as tendon cells for the APM. Our results reveal a functional role for basement membrane heterogeneity in tissue patterning. PAPERCLIP:..
  38. ncbi Direct binding of Reelin to VLDL receptor and ApoE receptor 2 induces tyrosine phosphorylation of disabled-1 and modulates tau phosphorylation
    T Hiesberger
    Department of Molecular Genetics, UT Southwestern Dallas, Texas 75235, USA
    Neuron 24:481-9. 1999
    ..Taken together, these findings suggest that Reelin acts via VLDLR and ApoER2 to regulate Disabled-1 tyrosine phosphorylation and microtubule function in neurons...
  39. ncbi Neuronal position in the developing brain is regulated by mouse disabled-1
    B W Howell
    Fred Hutchinson Cancer Research Center, Seattle, Washington 98109, USA
    Nature 389:733-7. 1997
    ..Because mDab1 is expressed in wild-type cortical neurons, and Reelin expression is normal in mdab1 mutants, mDab1 may be part of a Reelin-regulated or parallel pathway that controls the final positioning of neurons...
  40. pmc Frequency of Usher syndrome in two pediatric populations: Implications for genetic screening of deaf and hard of hearing children
    William J Kimberling
    Department of Genetics, Boys Town National Research Hospital, Omaha, Nebraska 68131, USA
    Genet Med 12:512-6. 2010
    ....
  41. ncbi Polymerization of type I and III collagens is dependent on fibronectin and enhanced by integrins alpha 11beta 1 and alpha 2beta 1
    Teet Velling
    Institute of Medical Biochemistry and Microbiology, 75123 Uppsala, Sweden
    J Biol Chem 277:37377-81. 2002
    ..Thus, collagen deposition is regulated by the cells, both indirectly through integrin alpha(5)beta(1)-dependent polymerization of fibronectin and directly through collagen-binding integrins...
  42. ncbi COMP acts as a catalyst in collagen fibrillogenesis
    Krisztina Halász
    Department of Experimental Medical Science, Lund University, SE 221 84 Lund, Sweden
    J Biol Chem 282:31166-73. 2007
    ..Thus, COMP appears to catalyze the fibril formation by promoting early association of collagen molecules leading to increased rate of fibrillogenesis and more distinct organization of the fibrils...
  43. ncbi Scrambler and yotari disrupt the disabled gene and produce a reeler-like phenotype in mice
    M Sheldon
    Department of Developmental Neurobiology, St Jude Children s Research Hospital, Memphis, Tennessee 38105, USA
    Nature 389:730-3. 1997
    ..The similar phenotypes of reeler, scrambler, yotari and mdab1 null mice indicate that Reelin and mDab1 function as signalling molecules that regulate cell positioning in the developing brain...
  44. ncbi Regulation of fibroblastic growth factor 23 expression but not degradation by PHEX
    Shiguang Liu
    Department of Medicine, Center for Bone and Mineral Disorders, Duke University Medical Center, Durham, North Carolina 27710, USA
    J Biol Chem 278:37419-26. 2003
    ....
  45. ncbi Targeted disruption of the biglycan gene leads to an osteoporosis-like phenotype in mice
    T Xu
    Craniofacial and Skeletal Diseases Branch, National Institute of Dental Research, National Institutes of Health, Bethesda, Maryland 20892, USA
    Nat Genet 20:78-82. 1998
    ..These mice may serve as an animal model to study the role of ECM proteins in osteoporosis...
  46. ncbi The DFNB31 gene product whirlin connects to the Usher protein network in the cochlea and retina by direct association with USH2A and VLGR1
    Erwin Van Wijk
    Department of Otorhinolaryngology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    Hum Mol Genet 15:751-65. 2006
    ..Our data show that whirlin is connected to the dynamic Usher protein interactome and indicate that whirlin has a pleiotropic function in both the retina and the inner ear...
  47. ncbi EFEMP1 expression promotes in vivo tumor growth in human pancreatic adenocarcinoma
    Hendrik Seeliger
    Munich University Medical Center, Department of Surgery, Munich, Germany
    Mol Cancer Res 7:189-98. 2009
    ..Hence, EFEMP1 is a promising candidate for assessing prognosis and individualizing therapy in a clinical tumor setting...
  48. ncbi SOX9 enhances aggrecan gene promoter/enhancer activity and is up-regulated by retinoic acid in a cartilage-derived cell line, TC6
    I Sekiya
    Department of Molecular Pharmacology, Medical Research Institute, Tokyo 101, Japan
    J Biol Chem 275:10738-44. 2000
    ..These observations indicate that SOX9 enhances aggrecan promoter activity and that its expression is up-regulated by RA in TC6 cells...
  49. pmc Interaction of reelin with amyloid precursor protein promotes neurite outgrowth
    HYANG SOOK HOE
    Department of Neuroscience, Georgetown University Medical Center, Washington, DC 20057 1464, USA
    J Neurosci 29:7459-73. 2009
    ..Addition of an alpha3beta1 integrin antibody prevented APP and Reelin-induced neurite outgrowth. These findings demonstrate that Reelin interacts with APP, potentially having important effects on neurite development...
  50. pmc Mutational screening of the USH2A gene in Spanish USH patients reveals 23 novel pathogenic mutations
    Gema García-García
    Grupo de Investigación en Enfermedades Neurosensoriales, Instituto de Investigación Sanitaria IIS La Fe, Valencia, Spain
    Orphanet J Rare Dis 6:65. 2011
    ..Among the three genes implicated, mutations in the USH2A gene account for 74-90% of the USH2 cases...
  51. pmc Missense mutation in the USH2A gene: association with recessive retinitis pigmentosa without hearing loss
    C Rivolta
    Ocular Molecular Genetics Institute and the Berman Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, MA, 02114, USA
    Am J Hum Genet 66:1975-8. 2000
    ..This single mutation was found in 4.5% of 224 patients with recessive RP, suggesting that USH2A could cause more cases of nonsyndromic recessive RP than does any other gene identified to date...
  52. ncbi EFEMP1 as a novel DNA methylation marker for prostate cancer: array-based DNA methylation and expression profiling
    Yong June Kim
    Department of Urology, College of Medicine, Chungbuk National University, Cheongju, South Korea
    Clin Cancer Res 17:4523-30. 2011
    ..The aim of the present study was to identify novel methylation markers in prostate cancer (PCa) by microarray analysis and to test whether these markers could discriminate normal and PCa cells...
  53. pmc RHAMM (CD168) is overexpressed at the protein level and may constitute an immunogenic antigen in advanced prostate cancer disease
    Kilian M Gust
    Department of Urology, University of Ulm, Ulm, Germany
    Neoplasia 11:956-63. 2009
    ..Acknowledging the proven immunogenic effects of RHAMM in leukemia, this antigen is intriguing as a therapeutic target in far-advanced CaP...
  54. ncbi Reeler/Disabled-like disruption of neuronal migration in knockout mice lacking the VLDL receptor and ApoE receptor 2
    M Trommsdorff
    Department of Molecular Genetics, University of Texas Southwestern Medical Center, Dallas 75235 9046, USA
    Cell 97:689-701. 1999
    ..These findings suggest that VLDLR and ApoER2 participate in transmitting the extracellular Reelin signal to intracellular signaling processes initiated by mDab1...
  55. ncbi Non-USH2A mutations in USH2 patients
    Thomas Besnard
    CHU Montpellier, Laboratoire de Genetique Moleculaire, Montpellier, France
    Hum Mutat 33:504-10. 2012
    ..In effect, these three genes account for the vast majority of USH2 patients and their analysis provide a robust pathway for routine molecular diagnosis...
  56. ncbi Reelin-mediated signaling locally regulates protein kinase B/Akt and glycogen synthase kinase 3beta
    Uwe Beffert
    Department of Molecular Genetics, Department of Cell Biology, University of Texas Southwestern Medical Center, Dallas, TX 75390 9046, USA
    J Biol Chem 277:49958-64. 2002
    ....
  57. pmc Proteomics reveals drastic increase of extracellular matrix proteins collagen and dermatopontin in the aged mdx diaphragm model of Duchenne muscular dystrophy
    Steven Carberry
    Department of Biology, National University of Ireland, Maynooth, Kildare, Republic of Ireland
    Int J Mol Med 30:229-34. 2012
    ..Aging in the mdx diaphragm seems to be associated with a drastic increase in the extracellular matrix proteins, collagen and dermatopontin, the molecular chaperone αB-crystallin, and the intermediate ..
  58. ncbi Usher syndrome (sensorineural deafness and retinitis pigmentosa): pathogenesis, molecular diagnosis and therapeutic approaches
    Crystel Bonnet
    Institut de la Vision, Paris, France
    Curr Opin Neurol 25:42-9. 2012
    ..In this review, we pinpoint new insights regarding the molecular mechanisms defective in this syndrome, its molecular diagnosis and prospective therapies...
  59. ncbi Fibulin-3 is associated with tumour progression and a poor prognosis in nasopharyngeal carcinomas and inhibits cell migration and invasion via suppressed AKT activity
    Chung Feng Hwang
    Department of Otolaryngology, Chang Gung Memorial Hospital Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan
    J Pathol 222:367-79. 2010
    ..Thus, lower fibulin-3 expression is an important indicator of poor survival. It may also contribute to the development of new therapeutic strategies to block the PI3K/AKT pathway in NPC cancer cells...
  60. ncbi A mechanism for inside-out lamination in the neocortex
    Jonathan A Cooper
    Division of Basic Sciences, Fred Hutchinson Cancer Research Center, Seattle, WA 98109, USA
    Trends Neurosci 31:113-9. 2008
    ..This 'detach and go' model explains many aspects of inside-out lamination, defects in the Reeler mutant and results of recent genetic and in utero experiments...
  61. pmc Mutations in the TGFβ binding-protein-like domain 5 of FBN1 are responsible for acromicric and geleophysic dysplasias
    Carine Le Goff
    Department of Genetics, Universite Paris Descartes, Unité Institut National de la Santé et de la Recherche Médicale, Hopital Necker Enfants Malades, Paris, France
    Am J Hum Genet 89:7-14. 2011
    ..Although enhanced TGFβ signaling caused by FBN1 mutations can trigger either Marfan syndrome or GD and AD, our findings support the fact that TB5 mutations in FBN1 are responsible for short stature phenotypes...
  62. pmc Human ductal adenocarcinomas of the pancreas express extracellular matrix proteins
    M Lohr
    Department of Medicine I, University of Erlangen Nuremberg, Germany
    Br J Cancer 69:144-51. 1994
    ..tumours from human ductal adenocarcinomas of the pancreas were examined for the expression of extracellular matrix proteins (ECM), using cDNA probes and antibodies to collagen types I, III and IV, vitronectin, fibronectin, ..
  63. ncbi Six genes expressed in bones and teeth encode the current members of the SIBLING family of proteins
    Larry W Fisher
    Matrix Biochemistry Unit, Craniofacial and Skeletal Diseases Branch, NIDCR, NIH, HHS, Bethesda, Maryland 20892 4320, USA
    Connect Tissue Res 44:33-40. 2003
    ..Two possible mechanisms for inhibiting the lytic pathway of alternate complement are presented...
  64. pmc Proteomics characterization of extracellular space components in the human aorta
    Athanasios Didangelos
    King s British Heart Foundation Centre, King s College, London, United Kingdom
    Mol Cell Proteomics 9:2048-62. 2010
    ....
  65. pmc Analysis of TGFBI gene mutations in Chinese patients with corneal dystrophies and review of the literature
    Juhua Yang
    Biomedical Engineering Center, Fujian Medical University, Fuzhou, Fujian, China
    Mol Vis 16:1186-93. 2010
    ..To analyze human transforming growth factor b-induced (TGFBI) gene mutations in Chinese patients with corneal dystrophies (CDs)...
  66. ncbi Hearts and bones: shared regulatory mechanisms in heart valve, cartilage, tendon, and bone development
    Joy Lincoln
    Division of Molecular Cardiovascular Biology, MLC 7020, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    Dev Biol 294:292-302. 2006
    ..This review will focus on the regulatory hierarchies that control normal and abnormal heart valve development in parallel with other connective tissue cell types...
  67. ncbi Fibromodulin-null mice have abnormal collagen fibrils, tissue organization, and altered lumican deposition in tendon
    L Svensson
    Department of Cell and Molecular Biology, University of Lund, P O Box 94, S 221 00 Lund, Sweden
    J Biol Chem 274:9636-47. 1999
    ..These results demonstrate a role for fibromodulin in collagen fibrillogenesis and suggest that the orchestrated action of several leucine-rich repeat glycoproteins/proteoglycans influence the architecture of collagen matrices...
  68. ncbi Autosomal recessive lissencephaly with cerebellar hypoplasia is associated with human RELN mutations
    S E Hong
    Division of Neurogenetics, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Institutes of Medicine, Boston, Massachusetts, USA
    Nat Genet 26:93-6. 2000
    ....
  69. pmc Extracellular matrix protein betaig-h3/TGFBI promotes metastasis of colon cancer by enhancing cell extravasation
    Chaoyu Ma
    Department of Pharmacology and Cancer Biology, Duke University Medical Center, Durham, North Carolina 27710, USA
    Genes Dev 22:308-21. 2008
    ..Thus, cancers associated with overexpression of betaig-h3 may have an increased metastatic potential, leading to poor prognosis in cancer patients...
  70. pmc RHAMM, a receptor for hyaluronan-mediated motility, compensates for CD44 in inflamed CD44-knockout mice: a different interpretation of redundancy
    Shlomo Nedvetzki
    The Lautenberg Center for General and Tumor Immunology, Hebrew University Hadassah Medical School, Jerusalem 91120, Israel
    Proc Natl Acad Sci U S A 101:18081-6. 2004
    ..This model enlightens several aspects of molecular redundancy, which is widely discussed in many scientific circles, but the processes are still ill defined...
  71. ncbi Genetic deletion of receptor for hyaluronan-mediated motility (Rhamm) attenuates the formation of aggressive fibromatosis (desmoid tumor)
    Cornelia Tolg
    Departments of Oncology and Biochemistry, The University of Western Ontario, Canada
    Oncogene 22:6873-82. 2003
    ....
  72. ncbi Negative regulation of transforming growth factor-beta by the proteoglycan decorin
    Y Yamaguchi
    Cancer Research Center, La Jolla Cancer Research Foundation, California 92037
    Nature 346:281-4. 1990
    ..As transforming growth factor-beta induces synthesis of decorin in many cell types, our results suggest that decorin may be a component of a feedback system regulating cell growth...
  73. ncbi A syndrome of joint laxity and impaired tendon integrity in lumican- and fibromodulin-deficient mice
    Karl J Jepsen
    Department of Orthopedics, Mount Sinai School of Medicine, New York, New York 10029, USA
    J Biol Chem 277:35532-40. 2002
    ..This study demonstrates that the collagen fibril-modifying proteoglycans, lumican and fibromodulin, are candidate genes and key players in the pathogenesis of certain types of Ehlers-Danlos syndrome and other connective tissue disorders...
  74. ncbi Homozygosity for a missense mutation in fibulin-5 (FBLN5) results in a severe form of cutis laxa
    Bart Loeys
    Center for Medical Genetics, Ghent University Hospital, Belgium
    Hum Mol Genet 11:2113-8. 2002
    ..As such, we provide evidence that a genetic defect in fibulin-5 (FBLN5, also known as EVEC or DANCE) is responsible for a recessive form of cutis laxa in humans...
  75. ncbi Modulation of synaptic plasticity and memory by Reelin involves differential splicing of the lipoprotein receptor Apoer2
    Uwe Beffert
    Department of Molecular Genetics, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA
    Neuron 47:567-79. 2005
    ..Thus, alternative splicing of Apoer2, a novel component of the NMDA receptor complex, controls the modulation of NMDA receptor activity, synaptic neurotransmission, and memory by Reelin...
  76. ncbi Gene and protein expression changes in human trabecular meshwork cells treated with transforming growth factor-beta
    Xiujun Zhao
    Section on Aging and Ocular Disease, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    Invest Ophthalmol Vis Sci 45:4023-34. 2004
    ..To determine the genomic and proteomic expression changes in human trabecular meshwork cells when they are treated with transforming growth factor (TGF)-beta...
  77. ncbi Transforming growth factor-beta1 induces desmoplasia in an experimental model of human pancreatic carcinoma
    M Lohr
    Department of Medicine, University of Rostock, Germany
    Cancer Res 61:550-5. 2001
    ..This effect cannot only be attributed to direct effects of TGF-beta1 but also results from the up-regulation of several other factors including collagen type I, connective tissue growth factor, and platelet-derived growth factor...
  78. pmc RHAMM is a centrosomal protein that interacts with dynein and maintains spindle pole stability
    Christopher A Maxwell
    Department of Oncology, University of Alberta Cross Cancer Institute, Edmonton Alberta Canada T6G 1Z2
    Mol Biol Cell 14:2262-76. 2003
    ..Moreover, we provide a potential mechanism for this function in that RHAMM may cross-link centrosomal microtubules, through a direct interaction with microtubules and an association with dynein...
  79. ncbi Update on Usher syndrome
    Zubin Saihan
    UCL Institute of Ophthalmology and Moorfields Eye Hospital, London, UK
    Curr Opin Neurol 22:19-27. 2009
    ..The present review addresses the mechanisms, genetics and pathogenesis of Usher syndrome...
  80. ncbi Fyn tyrosine kinase is a critical regulator of disabled-1 during brain development
    Lionel Arnaud
    Fred Hutchinson Cancer Research Center, 1100 Fairview Avenue North, 98109, Seattle, WA, USA
    Curr Biol 13:9-17. 2003
    ..Mutations in Dab1, its phosphorylation sites, Reelin, or the Reelin receptors cause a common phenotype. However, the molecular mechanism whereby Reelin regulates Dab1 tyrosine phosphorylation is poorly understood...
  81. pmc The extracellular matrix protein mindin serves as an integrin ligand and is critical for inflammatory cell recruitment
    Wei Jia
    Department of Immunology, Duke University Medical Center, Durham, NC 27710, USA
    Blood 106:3854-9. 2005
    ..Our results suggest that mindin serves as a novel ligand for integrins and mindin-integrin interactions are critical for inflammatory cell recruitment in vivo...
  82. ncbi Differential expression of bone matrix regulatory proteins in human atherosclerotic plaques
    C R Dhore
    Department of Pathology, Cardiovascular Research Institute Maastricht, University of Maastricht, Netherlands
    Arterioscler Thromb Vasc Biol 21:1998-2003. 2001
    ..The expression pattern of both OPG and OPGL during atherogenesis might suggest a regulatory role of these proteins not only in osteoclastogenesis but also in atherosclerotic calcification...
  83. ncbi Lipoprotein receptors in the nervous system
    Joachim Herz
    Department of Molecular Genetics, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, Texas 75390, USA
    Annu Rev Biochem 71:405-34. 2002
    ....
  84. pmc Overexpression of HTRA1 leads to ultrastructural changes in the elastic layer of Bruch's membrane via cleavage of extracellular matrix components
    Sarah Vierkotten
    Center of Ophthalmology, University of Cologne, Cologne, Germany
    PLoS ONE 6:e22959. 2011
    ..It highlights the importance of ECM related proteins in the development of AMD and links HTRA1 to other AMD risk genes such as fibulin 5, fibulin 6, ARMS2 and TIMP3...
  85. pmc Decreased chondrocyte proliferation and dysregulated apoptosis in the cartilage growth plate are key features of a murine model of epiphyseal dysplasia caused by a matn3 mutation
    Matthew P Leighton
    Wellcome Trust Centre for Cell Matrix Research, Faculty of Life Sciences, University of Manchester, Michael Smith Building, Oxford Road, Manchester M13 9PT, UK
    Hum Mol Genet 16:1728-41. 2007
    ....
  86. pmc Receptor clustering is involved in Reelin signaling
    Vera Strasser
    Max F Perutz Laboratories, University Departments at the Vienna Biocenter, Department of Medical Biochemistry, University of Vienna, Vienna, Austria
    Mol Cell Biol 24:1378-86. 2004
    ....
  87. ncbi Usher syndrome type 2 caused by activation of an USH2A pseudoexon: implications for diagnosis and therapy
    Christel Vache
    CHU Montpellier, Laboratoire de Genetique Moleculaire, Montpellier, France
    Hum Mutat 33:104-8. 2012
    ..Finally, this mutation, which would not have been found by whole-exome sequencing, could offer, for the first time in USH, the possibility of therapeutic correction by antisense oligonucleotides (AONs)...
  88. ncbi The expression of EFEMP1 in cervical carcinoma and its relationship with prognosis
    Song En-lin
    Biomedical Engineering Center, Department of Anatomy, the Zhongshan School of Medicine, Sun Yat Sen University, Guangzhou, Guangdong 510080, China
    Gynecol Oncol 117:417-22. 2010
    ..The study was to explore the role of EFEMP1 protein in angiogenesis and its relationship with prognosis of cervical carcinoma...
  89. pmc Crystal structure of the dimeric protein core of decorin, the archetypal small leucine-rich repeat proteoglycan
    Paul G Scott
    Department of Biochemistry and Alberta Synchrotron Institute, University of Alberta, Edmonton, Alberta, Canada T6G 2H7
    Proc Natl Acad Sci U S A 101:15633-8. 2004
    ..with a variety of important biological functions that are mediated by its interactions with extracellular matrix proteins, cytokines, and cell surface receptors...
  90. pmc Immune response to biologic scaffold materials
    Stephen F Badylak
    McGowan Institute for Regenerative Medicine, University of Pittsburgh, Pittsburgh, PA 15219, United States
    Semin Immunol 20:109-16. 2008
    ..This manuscript provides an overview of studies that have evaluated the immune response to biologic scaffold materials and variables that affect this response...
  91. pmc Aberrant accumulation of EFEMP1 underlies drusen formation in Malattia Leventinese and age-related macular degeneration
    Lihua Y Marmorstein
    Cole Eye Institute, and Department of Cell Biology, Lerner Research Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA
    Proc Natl Acad Sci U S A 99:13067-72. 2002
    ..These data present evidence that misfolding and aberrant accumulation of EFEMP1 may cause drusen formation and cellular degeneration and play an important role in the etiology of both ML and AMD...
  92. ncbi Warfarin causes rapid calcification of the elastic lamellae in rat arteries and heart valves
    P A Price
    Department of Biology, University of California, San Diego, La Jolla 92093 0368, USA
    Arterioscler Thromb Vasc Biol 18:1400-7. 1998
    ..The results indicate that the improved warfarin plus vitamin K treatment protocol developed in this study should provide a useful model to investigate the role of MGP in preventing calcification of arteries and heart valves...
  93. ncbi Role of calcification inhibitors in the pathogenesis of vascular calcification in chronic kidney disease (CKD)
    Sharon M Moe
    Indiana University School of Medicine, Indianapolis, Indiana 46202, USA
    Kidney Int 67:2295-304. 2005
    ..This suggests protective mechanisms, or naturally occurring inhibitors, of calcification may be important...
  94. ncbi Spectrum of USH2A mutations in Scandinavian patients with Usher syndrome type II
    Bo Dreyer
    Department of Medical Genetics, Institute of Clinical Medicine, University of Tromsø, NO 9037 Tromsø, Norway
    Hum Mutat 29:451. 2008
    ..2%) families the USH phenotype could be explained by mutations in the USH3A gene. The results presented here provide a comprehensive picture of the genetic aetiology of Usher syndrome type IIA in Scandinavia as it is known to date...
  95. pmc Proteoglycans and more--from molecules to biology
    Dick Heinegard
    Department of Clinical Sciences, Section for Rheumatology, Molecular Skeletal Biology, Lund University, Lund, Sweden
    Int J Exp Pathol 90:575-86. 2009
    ..Those fragments released can be specifically detected employing antibodies specific to the cleavage site and used to diagnose and monitor e.g. joint disease at early stages...
  96. pmc Sox9 is required for precursor cell expansion and extracellular matrix organization during mouse heart valve development
    Joy Lincoln
    Department of Molecular and Cellular Pharmacology, Leonard M Miller School of Medicine, University of Miami, Miami, FL 33101, USA
    Dev Biol 305:120-32. 2007
    ..These data indicate that Sox9 is required for early and late stages of valvulogenesis and identify a potential role for Sox9 in valve disease mechanisms...
  97. pmc Extracellular matrix mineralization is regulated locally; different roles of two gla-containing proteins
    Monzur Murshed
    Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, Rm S921, Houston, TX 77030, USA
    J Cell Biol 165:625-30. 2004
    ..These results indicate that ECMM is regulated locally in animals and uncover a striking disparity of function between proteins sharing identical structural motifs...
  98. ncbi Disabled-1 acts downstream of Reelin in a signaling pathway that controls laminar organization in the mammalian brain
    D S Rice
    Department of Developmental Neurobiology, St Jude Children s Research Hospital, Memphis, Tennessee 38105, USA
    Development 125:3719-29. 1998
    ..This suggests that Dab1 accumulates in the absence of a Reln-evoked signal. Taken together, these results indicate that Dab1 functions downstream of Reln in a signaling pathway that controls cell positioning in the developing brain...
  99. ncbi Molecular and in silico analyses of the full-length isoform of usherin identify new pathogenic alleles in Usher type II patients
    David Baux
    Centre Hospitalier Universitaire CHU Montpellier, Laboratoire de Genetique Moleculaire, Montpellier, France
    Hum Mutat 28:781-9. 2007
    ..We have identified a previously unrecognized cysteine rich structural domain, containing 12 dicysteine repeats, and show that three missense mutations result in the loss of one of a pair of the defining cysteine-cysteine pairs...
  100. ncbi Beta1-class integrins regulate the development of laminae and folia in the cerebral and cerebellar cortex
    D Graus-Porta
    Friedrich Miescher Institute, Maulbeerstr 66, 4058, Basel, Switzerland
    Neuron 31:367-79. 2001
    ..The phenotype of the beta1-deficient mice resembles pathological changes observed in human cortical dysplasias, suggesting that defective integrin-mediated signal transduction contributes to the development of some of these diseases...
  101. ncbi Mechanisms for asporin function and regulation in articular cartilage
    Masahiro Nakajima
    Laboratory for Bone and Joint Diseases, SNP Research Center, RIKEN, 4 6 1 Shirokanedai, Minato ku, Tokyo 108 8639, Japan
    J Biol Chem 282:32185-92. 2007
    ..Our results provide a basis for elucidating the role of asporin in the molecular pathogenesis of OA...

Research Grants71

  1. INSULIN RECEPTORS AND THE GLUCOSE TRANSPORT SYSTEM
    JERROLD MICHAEL OLEFSKY; Fiscal Year: 2013
    ..To- wards this end, we have identified novel secretory and extracellular matrix proteins (biglycan and galectin 3) and a new GPCR (GPR105) which are differentially highly expressed in CD11c ..
  2. MECHANISMS OF RADIORESISTANCE AND CELL CYCLE PROGRESSION
    Howard B Lieberman; Fiscal Year: 2013
    ..that reflect metastasis, including migration towards fibronectin, invasion, cell adhesion to various extracellular matrix proteins, and metastatic behavior in an in vivo model...
  3. Metabolic regulation of diabetic nephropathy
    Karen Block; Fiscal Year: 2013
    ..Histologically, diabetic nephropathy is characterized as an excessive accumulation of extracellular matrix proteins (Fibronectin and Collagens)...
  4. IGFBP-5 and Hsp70 in Extracellular Matrix Homeostasis and Fibrosis
    Kristen L Veraldi; Fiscal Year: 2013
    ..IGFBP-5 induces expression of the major extracellular matrix proteins fibronectin and collagen I...
  5. Mechanisms of Airway Smooth Muscle Hypertrophy
    Marc B Hershenson; Fiscal Year: 2013
    ..cells express high levels of mRNAs encoding matricellular proteins, a subfamily of non-structural extracellular matrix proteins that regulate cell-matrix interactions and fiber deposition...
  6. Genetic Analysis of FAK Activity
    David D Schlaepfer; Fiscal Year: 2012
    Genetic Analysis of FAK Activity Binding interactions between extracellular matrix proteins such as fibronectin and integrins play fundamental roles during development by controlling cell adhesion, motility, and survival...
  7. Role of DDR2 in OA-like Pathogenesis in Osteochondrodysplasias
    Yefu Li; Fiscal Year: 2010
    ..changes in articular cartilage that are associated with osteochondrodysplasias caused by mutations in extracellular matrix proteins. This grant application is based on findings that (1) type IX collagen-deficient (Col9a1-/-) or type ..
  8. Role of Biophysical Cues in the Pathogenesis of Glaucoma
    Paul Russell; Fiscal Year: 2013
    ..We would focus on extracellular matrix proteins and remodeling of matrix by the HTM cells when they are supplied with biophysical cues (compliance and ..
  9. Harness Germinal Matrix Hemorrhage
    John H Zhang; Fiscal Year: 2013
    ..We hypothesize that the activation of PARs by thrombin will cause the overproduction of extracellular matrix proteins, via mTOR activation, thus obstructing CSF drainage and inducing hydrocephalus...
  10. PTH-RELATED PEPTIDE AND VIT D IN PROSTATE CANCER GROWTH
    Miriam Falzon; Fiscal Year: 2009
    ..We show that PTHrP overexpression increases prostate cancer cell (a) proliferation;(b) adhesion to the extracellular matrix proteins collagen types I and IV, laminin, and fibronectin;(c) migration onto laminin and collagen type I;(d) ..
  11. The Role of Fibrocytes in Liver Fibrosis
    Tatiana Kisseleva; Fiscal Year: 2012
    ..Chronic liver injury results in hepatic fibrosis, which is characterized by extensive deposition of extracellular matrix proteins (ECM), increased levels of TGF-[unreadable]1 and circulating endotoxin (lipopolysaccharide, LPS), and ..
  12. Role for tissue-type plasminogen activator in suppression of hepatic fibrosis
    Liang I Kang; Fiscal Year: 2013
    ..Upon activation, they transform into myofibroblast-like cells that promote the accumulation of extracellular matrix proteins, eventually distorting liver architecture and impairing liver function...
  13. Transglutaminase 2 S-Nitrosylation: Role in Age-Related Vascular Stiffness
    Dan E Berkowitz; Fiscal Year: 2013
    ..in vascular endothelial, smooth muscle cells, and fibroblasts, enzymatically form cross-links between extracellular matrix proteins, and may contribute to this pathobiology...
  14. Very Late Antigen Integrin Activity on T Cells
    Yoji Shimizu; Fiscal Year: 2010
    ..a vital role in cell function and development by mediating the adhesion of cells to other cells and to extracellular matrix proteins. In the immune system, the functional activity of integrins expressed on T lymphocytes is dynamically ..
  15. Mechanisms of adhesion and growth control in stem cells
    Reuben Kapur; Fiscal Year: 2010
    ..The BM microenvironment consists of extracellular matrix proteins as well as cells such as osteoblasts and stromal cells which contribute to the stem/progenitor cell ..
  16. Dysregulated Pathways in Pulmonary Fibrosis
    Keith C Olsen; Fiscal Year: 2012
    ..These myofibroblasts are the major effector cell type in fibrosis producing excess amounts of the extracellular matrix proteins including collagen and fibronectin...
  17. Zeiss 2-photon (2P) LSM780 laser scanning confocal microscope
    Moshe Levi; Fiscal Year: 2013
    ..so called epithelial to mesenchymal transformation is also associated with accumulation of fibrillary extracellular matrix proteins. In contrast in advanced atherosclerosis activation of tissue matrix metalloproteinases can result in ..
  18. Identification of Immune modulators associated with JC virus replication
    Jennifer Gordon; Fiscal Year: 2013
    ..We have also detected upregulation of cytokines, extracellular matrix proteins, and other cellular factors by microarray analysis of JCV infected astrocytes and immunohistochemistry ..
  19. Discovering Microenvironments Promoting Pathological EMT using Array Technology
    Naira Serobyan; Fiscal Year: 2012
    ..cell array screening system to identify physiologically relevant microenvironments consisting of extracellular matrix proteins, growth factors, and signaling molecules that promote epithelial mesenchymal transition (EMT) of lung ..
  20. Regulation of neutrophil migration by Activated Protein C
    Minsoo Kim; Fiscal Year: 2010
    ..Our preliminary data show that rhAPC inhibits neutrophil adhesion and migration on extracellular matrix proteins by directly blocking leukocyte integrins (21 and 23 integrins) on neutrophil surface...
  21. Claudin-2, Claudin-12 and Cadherin-17: Novel Targets of Vitamin D Action
    ANGELA R PORTA; Fiscal Year: 2011
    ..Experiments proposed will give insight for the first time into the in vivo role of the extracellular matrix proteins claudin-2, claudin-12 and cadherin-17 in vitamin D mediated intestinal calcium transport in the ..
  22. Role of Beta 1 containing Integrins in Cutaneous Basement Membrane Organization
    Srikala Raghavan; Fiscal Year: 2010
    ..Basement membranes (BM) are composed of ordered arrays of characteristic extracellular matrix proteins and both the epidermis and the dermis contribute to the synthesis of basement membrane components...
  23. Mineralization of the matrix in disease and health
    PETER S ROWE; Fiscal Year: 2013
    ..Key players in this bone kidney axis are a group of related extracellular matrix proteins (SIBLINGs). These proteins all map to a tightly clustered region on chromosome 4q...
  24. Hit to Lead Optimization of Small Molecule Therapeutics for Muscular Dystrophy
    Bradley L Hodges; Fiscal Year: 2010
    ..such as dystrophin and plectin, membrane proteins such as the sarcoglycans and alpha dystroglycan, or extracellular matrix proteins such as laminin-211 and collagen VI...
  25. Enabling Strategies for Growing Collagenous Tissues
    LAURA E contact NIKLASON; Fiscal Year: 2010
    ..connective tissues "from scratch", then it is crucial to understand the deposition and remodeling of extracellular matrix proteins, particularly collagen and elastin...
  26. Phytochemicals &Metabolism in Pancreatic Diseases/Pandol, Stephen
    Vay Liang W Go; Fiscal Year: 2011
    ..We have recently demonstrated that both growth factors and extracellular matrix proteins activate prosurvival pathways in pancreatic cancer cells through their ability to activate systems ..
  27. Self-assembling Biomimetic Hydrogels with Bioadhesive Properties for Intervertebr
    ANDREA JENNIFER VERNENGO; Fiscal Year: 2013
    ..Schiff's base reaction, thus rendering the hydrogel bioadhesive upon contact with amines of the extracellular matrix proteins. However, the presence of the reactive aldehyde groups can compromise the viability of encapsulated ..
  28. Development of a Novel TGF-beta Antagonist for Treating Hepatic Fibrosis
    Jung S Huang; Fiscal Year: 2010
    ..characterized by inflammatory cell infiltration, fibroblast proliferation, and excessive deposition of extracellular matrix proteins in liver parenchyma. There are million people around the world affected by HF. In the U.S...
  29. Molecular and circuit-level synergies in decision-making after early-life cocaine
    SHANNON LEIGH GOURLEY; Fiscal Year: 2013
    ..Throughout, we will focus on [unreadable]1-integrin systems. [unreadable]1-integrin is a receptor for extracellular matrix proteins, and it is implicated in cocaine addiction in humans...
  30. Innate Immune Response and Intestinal Inflammation
    Sang Hoon Rhee; Fiscal Year: 2010
    Liver fibrosis, the accumulation of extracellular matrix proteins, occurs in most types of chronic liver disease...
  31. Molecular Mechanisms Controlling Homeostatic Cellular Excitability
    Tingting Wang; Fiscal Year: 2012
    ..Unraveling the function of extracellular matrix proteins in synaptic transmission and homeostatic plasticity could, therefore, be a critical step in developing ..
  32. High-throughput genetic & small-molecule screening for therapeutic modifiers
    Peter G Schultz; Fiscal Year: 2010
    ..is a widely expressed transmembrane glycoprotein that acts as a high- affinity receptor for both extracellular matrix proteins containing laminin-G domains and certain arenaviruses...
  33. Mechanism of Tgfbr2 in chondroprotection
    Rosa A Serra; Fiscal Year: 2013
    ..down-stream targets of TGF-[unreadable] that regulate post-translational processing of the major extracellular matrix proteins in articular cartilage...
  34. INTRINSICALLY DISORDERED PROTEINS IN BIOMINERALIZATION
    JANET M OLDAK; Fiscal Year: 2013
    ..We propose that disordered domains in the extracellular matrix proteins play substantial roles in biomineralization...
  35. Alternative splicing in the vascular response to pathological shear stress
    PATRICK ANDRIES MURPHY; Fiscal Year: 2013
    ..The extracellular matrix proteins underlying the endothelium dictat the inflammatory response to altered flow...
  36. Blocking toll-like receptor 4 signaling as therapy in hepatic fibrosis
    BERND G SCHNABL; Fiscal Year: 2013
    Liver fibrosis, the accumulation of extracellular matrix proteins, occurs in most types of chronic liver disease...
  37. PROTEOGLYCANS IN NORMAL AND SCARRED CORNEAS
    James L Funderburgh; Fiscal Year: 2012
    ..a common pathological feature is a marked alteration of corneal proteoglycans, a specialized group of extracellular matrix proteins modified with carbohydrate...
  38. Biomimetic micro-structured hydrogel scaffolds for tissue engineered heart valves
    Jennifer L West; Fiscal Year: 2013
    ..function of heart valves is made possible by the unique microstructural arrangement of fibrous extracellular matrix proteins within the valve leaflet tissue, but these valvular structure- function relationships have not been ..
  39. Effects of chronic alcohol consumption on cardiac inflammation and fibrosis
    BRITTANY ANN LAW; Fiscal Year: 2011
    ..into myofibroblasts, which actively participate in tissue remodeling through the secretion of extracellular matrix proteins. It appears that persistent inflammation induced by excessive alcohol consumption plays a role in the ..
  40. THE ROLE OF THE SECRETORY PATHWAY IN CRANIOFACIAL MORPHOGENESIS
    Ela W Knapik; Fiscal Year: 2010
    ..Because several pathological conditions like scar formation and fibrosis involve secretion of extracellular matrix proteins, finding out how intracellular transport of large cargo is regulated could offer new ways to handle ..
  41. Why is Fam83h critical for enamel formation?
    Jan C C Hu; Fiscal Year: 2013
    ..About 25% of all amelogenesis imperfecta (AI) cases are caused by the genes encoding four enamel extracellular matrix proteins, AMELX, ENAM, MMP20, and KLK4...
  42. Structure and Function of 3-O-sulfation in Heparan Sulfate
    Jeffrey D Esko; Fiscal Year: 2013
    ..DESCRIPTION (provided by applicant): Heparan sulfate binds many growth factors, chemokines, morphogens, extracellular matrix proteins, enzymes and enzyme inhibitors via relatively short sulfated saccharide sequences...
  43. Integrin-Mediated Matrix Signaling in Liver Fibrosis
    Lester F Lau; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Liver fibrosis is the excessive accumulation of extracellular matrix proteins in response to chronic liver injuries, irrespective of the underlying etiology...
  44. Role of extracellular matrix proteins in Candida biofilm formation
    PRANAB KUMAR MUKHERJEE; Fiscal Year: 2010
    ....
  45. Searching for Novel Genes in Heparan Sulfate Biosynthesis
    Emylie Seamen; Fiscal Year: 2013
    ..various protein ligands that mediate cell proliferation, including growth factors, morphogens, other extracellular matrix proteins, enzymes, and enzyme inhibitors...
  46. Mechanism of Staphylocoagulase-activated Blood Clotting
    Paul E Bock; Fiscal Year: 2012
    ..ZAAP) family of SC homologs activate zymogens, cleave protein substrates, and bind to plasma and extracellular matrix proteins will be investigated...
  47. Cigarette Smoke induced Extracellular Matrix Remodeling in COPD
    Candace D Gildner; Fiscal Year: 2012
    ..Changes in the deposition and physiologic properties of individual extracellular matrix proteins in response to cigarette smoke have yet to be determined...
  48. Human islet transplantation on microporous scaffolds
    Lonnie D Shea; Fiscal Year: 2010
    ..of scaffold pore size and thickness on islet engraftment, 2) to investigate the impact of presenting extracellular matrix proteins on the microporous scaffold on the engraftment and function of transplanted human islets, and 3) to ..
  49. Endovascular device and treatment for stabilizing aortic aneurysms
    MATTHEW FRANK OGLE; Fiscal Year: 2010
    ..In previous studies, we have shown that PGG binds to aortic tissue, protects extracellular matrix proteins such as elastin, mechanically strengthens the tissue, and is effective in stopping aneurysm growth/..
  50. STRUCTURE OF ORAL BACTERIAL ADHESINS
    Teresa Ruiz; Fiscal Year: 2012
    ..non-fimbrial adhesins to adhere to and invade into epithelial cells as well as interacting with extracellular matrix proteins. EmaA (Extracellular matrix protein adhesin-A) is a newly discovered non-fimbrial adhesin of A...
  51. Soft Hydrogels for Expansion of Quiescent Human MSCs
    Sarah Atzet; Fiscal Year: 2010
    ..The goal of this proposal is to test the feasibility of a simple system for covalent attachment of extracellular matrix proteins to the HyStem hydrogel and to tailor the protein composition and stiffness for modulating the ..
  52. Functional and Biological Phenotyping of Pediatric PH
    Robin Shandas; Fiscal Year: 2013
    ..pulmonary vessels stiffen, which have led to novel and interesting hypotheses regarding the role of extracellular matrix proteins in upstream vascular remodeling, mechanisms of healthy versus maladaptive remodeling, and differences ..
  53. Syndecan-2 and Clinical Progression of Idiopathic Pulmonary Fibrosis
    Ivan O Rosas; Fiscal Year: 2012
    ..family of heparan-sulfate proteoglycans, can bind multiple chemokines, cytokines, growth factors and extracellular matrix proteins. Syndecan-2 can bind to these molecules as a membrane bound or shed protein...
  54. The role of RPTPzeta in models of Congenital Muscular Dystrophies
    Russell T Matthews; Fiscal Year: 2013
    ..high affinity ligands for a number of developmentally important adhesion molecules, growth factors and extracellular matrix proteins. We hypothesize that the hypoglycosylation of RPTP6 due to disrupted O-mannosylation alters the ..
  55. The role of connective Tissue Growth Factor on BMP9-Induced Osteogenesis
    HUE HAN LUU; Fiscal Year: 2011
    ..a secreted protein which has four distinct modules (domains) that share structural homology to several extracellular matrix proteins. Each module may have one or more distinct functions...
  56. Biannual von Hippel Lindau Family Alliance Medical Meeting
    Eric Jonasch; Fiscal Year: 2011
    ..cilia centrosome cycle control, co-regulation of cell cycle via p53 protein, and management of extracellular matrix proteins, including collagen IV and fibronectin...
  57. Genome-Wide Analysis of Ion Channels Required For Mechanosensation
    William D Tracey; Fiscal Year: 2010
    ..The Mec10/Mec4 channel is the central component of a transduction complex that also involves extracellular matrix proteins. In bacteria, a simpler form of mechanotransduction involves a mechanosensitive channel of large ..
  58. Lens capsule and secondary cataract
    Ram H Nagaraj; Fiscal Year: 2013
    ..Glycation is a major chemical modifier of extracellular matrix proteins. The reaction occurs between protein amino groups and carbonyl compounds and leads to the formation of ..
  59. Control of fibroblast function by prostaglandin E2 and plasminogen activation
    MARC L PETERS-GOLDEN; Fiscal Year: 2013
    ..effector cell in this disorder is the myofibroblast, with an exuberant capacity for elaboration of extracellular matrix proteins such as collagen that comprise tissue scars...
  60. ENAMEL MATRIX PROTEIN INTERACTION
    MICHAEL LANSDELL PAINE; Fiscal Year: 2010
    ..goal of this proposed work is to characterize protein-protein interactions involving the enamel extracellular matrix proteins, as well as proteins integral to the plasma membrane of ameloblast cells, and characterize how these ..
  61. Local Radionuclide Delivery to Solid Tumors by Injectible Biopolymer
    Wenge Liu; Fiscal Year: 2013
    ..reversible crosslinking of ELP coacervates, and (2) binding of ELPs and their proteolytic fragments to extracellular matrix proteins. These genetically engineered ELPs will be conjugated to 131I, a therapeutic radioisotope, and infused ..
  62. Engineering an Atherosclerosis-Resistant Endothelium
    Martin A Schwartz; Fiscal Year: 2011
    ..but not on basement membrane proteins due to differential signaling by the integrins that bind these extracellular matrix proteins. Furthermore, FN is found at atherosclerosis-prone sites in vivo prior to other indications of disease,..
  63. MONONUCLEAR LEUKOCYTES AND IMMUNITY
    Samuel Silverstein; Fiscal Year: 2003
    ..proposed focus on functions of plasma membrane transporters, and of receptors for nucleotide and extracellular matrix proteins on these cells. The project has three aims: #1...
  64. ENVIRONMENTAL MODULATION OF FIBRINOLYSIS INHIBITORS
    STEVEN GONIAS; Fiscal Year: 1992
    ..however, in vivo, fibrinolysis probably occurs on the surfaces of fibrin, endothelial cells, and extracellular matrix proteins. Little is known about the function of fibrinolysis proteinase inhibitors in these environments...
  65. A Therapeutic Agent for Radiation-induced Lung Fibrosis
    SHUAN HUANG; Fiscal Year: 2009
    ..characterized by inflammatory cell infiltration, fibroblast proliferation, and excessive deposition of extracellular matrix proteins (ECM) in lung parenchyma. Globally, five million people are affected by lung fibrosis. In the U.S...
  66. STRUCTURAL AND MUTATIONAL ANALYSIS OF THE LAMA3 GENE
    Angela Christiano; Fiscal Year: 1999
    Laminins comprise a family to trimeric extracellular matrix proteins expressed in the basement membranes of many tissues, appearing first during the early stages of embryonic development, and later in a wide variety of tissue-specific ..
  67. PERIODONTAL LIGAMENT CELL AUTOCRINE FACTOR
    VICTOR TERRANOVA; Fiscal Year: 1993
    ..b>Extracellular matrix proteins and various polypeptide growth factors have been shown to stimulate both chemotaxis and proliferation ..
  68. Tyrosine Sulfation and Chemokine Receptor Function
    Kevin Moore; Fiscal Year: 2006
    ..to be tyrosine sulfated in man, including certain adhesion molecules, coagulation factors, serpins, extracellular matrix proteins, and hormones and some of these require sulfation for optimal function...
  69. Pathogenesis of Borrelia burgdorferi induced arthritis
    Linden Hu; Fiscal Year: 2006
    ..burgdorferi does not secrete any enzymes capable of digesting extracellular matrix proteins in the joint...
  70. Surface Modified Nanofibers for Nerve Regeneration
    JOSEPH COREY; Fiscal Year: 2009
    ..Most nerve guides produce inferior results, even when filled or coated with extracellular matrix proteins (ECM) or growth factors that promote neurite outgrowth, including laminin, regarded as the most potent ..
  71. REGULATION OF PROUROKINASE-MEDIATED FIBRINOLYSIS BY ZINC
    S Husain; Fiscal Year: 1990
    ..activator (tPA) are believed to play a role in thrombolysis as well as localized degradation of extracellular matrix proteins. There has been a keen interest in these plasminogen activators as therapeutic agents for dissolving ..