collagen type iv

Summary

Summary: A non-fibrillar collagen found in the structure of BASEMENT MEMBRANE. Collagen type IV molecules assemble to form a sheet-like network which is involved in maintaining the structural integrity of basement membranes. The predominant form of the protein is comprised of two alpha1(IV) subunits and one alpha2(IV) subunit, however, at least six different alpha subunits can be incorporated into the heterotrimer.

Top Publications

  1. ncbi Genetics and clinical characteristics of keratoconus
    Mirna Stabuc-Silih
    University Eye Clinic, Ljubljana Medical Center, 1000 Ljubljana, Slovenia
    Acta Dermatovenerol Alp Pannonica Adriat 19:3-10. 2010
  2. ncbi Breaching the basement membrane: who, when and how?
    R Grant Rowe
    Division of Molecular Medicine and Genetics, Department of Internal Medicine, The Life Sciences Institute, University of Michigan, 210 Washtenaw, Ann Arbor, MI 48109 2216, USA
    Trends Cell Biol 18:560-74. 2008
  3. ncbi Collagen IV is essential for basement membrane stability but dispensable for initiation of its assembly during early development
    Ernst Poschl
    Department of Experimental Medicine I, University Erlangen Nurnberg, 91054 Erlangen, Germany
    Development 131:1619-28. 2004
  4. pmc Shaping cells and organs in Drosophila by opposing roles of fat body-secreted Collagen IV and perlecan
    Jose Carlos Pastor-Pareja
    Howard Hughes Medical Institute, Department of Genetics, Yale University School of Medicine, 295 Congress Avenue, New Haven, CT 06519, USA
    Dev Cell 21:245-56. 2011
  5. ncbi Distinct target-derived signals organize formation, maturation, and maintenance of motor nerve terminals
    Michael A Fox
    Department of Molecular and Cellular Biology, Harvard University, Cambridge, MA 02138, USA
    Cell 129:179-93. 2007
  6. ncbi Mutations in Col4a1 cause perinatal cerebral hemorrhage and porencephaly
    Douglas B Gould
    Howard Hughes Medical Institute, Bar Harbor, ME 04609, USA
    Science 308:1167-71. 2005
  7. pmc Physiological levels of tumstatin, a fragment of collagen IV alpha3 chain, are generated by MMP-9 proteolysis and suppress angiogenesis via alphaV beta3 integrin
    Yuki Hamano
    Center for Matrix Biology, Department of Medicine and Gastroenterology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02215, USA
    Cancer Cell 3:589-601. 2003
  8. ncbi Mammalian collagen IV
    Jamshid Khoshnoodi
    Department of Medicine, Vanderbilt University, Nashville, Tennessee 37232, USA
    Microsc Res Tech 71:357-70. 2008
  9. pmc Hemocyte-secreted type IV collagen enhances BMP signaling to guide renal tubule morphogenesis in Drosophila
    Stephanie Bunt
    Department of Zoology, University of Cambridge, UK
    Dev Cell 19:296-306. 2010
  10. pmc Quantitative trait loci influence renal disease progression in a mouse model of Alport syndrome
    Kaya L Andrews
    Renal Division, Department of Internal Medicine, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, Missouri 63110, USA
    Am J Pathol 160:721-30. 2002

Research Grants

  1. Role of Peroxidasin in Glomerular Matrix Homeostasis
    Gautam Bhave; Fiscal Year: 2013
  2. Matrix Otopathology
    MICHAEL ANNE NMI GRATTON; Fiscal Year: 2011
  3. Genetically testing interactions of ER and oxidative stresses in retinal disease
    Douglas Gould; Fiscal Year: 2013
  4. FEASIBILITY OF REPAIRING GBM DEFECTS IN VIVO
    Jeffrey H Miner; Fiscal Year: 2012
  5. Extracellular Scaffold Elasticity and Binding Sites in Acinar Differentiation
    Melinda Larsen; Fiscal Year: 2013
  6. Nicotine and Chronic Kidney Disease
    Edgar A Jaimes; Fiscal Year: 2010
  7. Invasive Behavior of Tumor Cells Producing Collagenase-1
    Constance E Brinckerhoff; Fiscal Year: 2013
  8. SIGNALING MECHANISMS IN REGULATION OF TUMOR ANGIOGENESIS
    DOMINIC E COSGROVE; Fiscal Year: 2012
  9. QD-BRET nanosensors for protease detection and imaging
    Jianghong Rao; Fiscal Year: 2012
  10. MOLECULAR ASPECTS OF ALPORT RENAL DISEASE PROGRESSION
    DOMINIC E COSGROVE; Fiscal Year: 2013

Detail Information

Publications314 found, 100 shown here

  1. ncbi Genetics and clinical characteristics of keratoconus
    Mirna Stabuc-Silih
    University Eye Clinic, Ljubljana Medical Center, 1000 Ljubljana, Slovenia
    Acta Dermatovenerol Alp Pannonica Adriat 19:3-10. 2010
    ....
  2. ncbi Breaching the basement membrane: who, when and how?
    R Grant Rowe
    Division of Molecular Medicine and Genetics, Department of Internal Medicine, The Life Sciences Institute, University of Michigan, 210 Washtenaw, Ann Arbor, MI 48109 2216, USA
    Trends Cell Biol 18:560-74. 2008
    ..Further characterization of the molecular mechanisms underlying BM transmigration should provide important insights into pathophysiologic tissue remodeling events and also enable the development of novel therapeutics...
  3. ncbi Collagen IV is essential for basement membrane stability but dispensable for initiation of its assembly during early development
    Ernst Poschl
    Department of Experimental Medicine I, University Erlangen Nurnberg, 91054 Erlangen, Germany
    Development 131:1619-28. 2004
    ....
  4. pmc Shaping cells and organs in Drosophila by opposing roles of fat body-secreted Collagen IV and perlecan
    Jose Carlos Pastor-Pareja
    Howard Hughes Medical Institute, Department of Genetics, Yale University School of Medicine, 295 Congress Avenue, New Haven, CT 06519, USA
    Dev Cell 21:245-56. 2011
    ..Our results uncover incorporation of Collagen IV and Perlecan into BMs as a major determinant of organ shape and animal form...
  5. ncbi Distinct target-derived signals organize formation, maturation, and maintenance of motor nerve terminals
    Michael A Fox
    Department of Molecular and Cellular Biology, Harvard University, Cambridge, MA 02138, USA
    Cell 129:179-93. 2007
    ..Thus, multiple target-derived signals permit discrete control of the formation, maturation, and maintenance of presynaptic specializations...
  6. ncbi Mutations in Col4a1 cause perinatal cerebral hemorrhage and porencephaly
    Douglas B Gould
    Howard Hughes Medical Institute, Bar Harbor, ME 04609, USA
    Science 308:1167-71. 2005
    ..We also show that COL4A1 mutations segregate with porencephaly in human families. Because not all mutant mice develop porencephaly, we propose that Col4a1 mutations conspire with environmental trauma in causing the disease...
  7. pmc Physiological levels of tumstatin, a fragment of collagen IV alpha3 chain, are generated by MMP-9 proteolysis and suppress angiogenesis via alphaV beta3 integrin
    Yuki Hamano
    Center for Matrix Biology, Department of Medicine and Gastroenterology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02215, USA
    Cancer Cell 3:589-601. 2003
    ..These results indicate that MMP-generated fragments of basement membrane collagen can have endogenous function as integrin-mediated suppressors of pathologic angiogenesis and tumor growth...
  8. ncbi Mammalian collagen IV
    Jamshid Khoshnoodi
    Department of Medicine, Vanderbilt University, Nashville, Tennessee 37232, USA
    Microsc Res Tech 71:357-70. 2008
    ..In this review article, we will summarize essential findings on the structural and functional relationships of different collagen IV chains and their roles in health and disease...
  9. pmc Hemocyte-secreted type IV collagen enhances BMP signaling to guide renal tubule morphogenesis in Drosophila
    Stephanie Bunt
    Department of Zoology, University of Cambridge, UK
    Dev Cell 19:296-306. 2010
    ..Such regulated interplay between cell-cell and cell-matrix interactions is likely to have wide relevance in organogenesis and congenital disease...
  10. pmc Quantitative trait loci influence renal disease progression in a mouse model of Alport syndrome
    Kaya L Andrews
    Renal Division, Department of Internal Medicine, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, Missouri 63110, USA
    Am J Pathol 160:721-30. 2002
    ..Analysis with Map Manager QT revealed QTLs linked to markers on chromosomes 9 and 16. A more detailed understanding of how these QTLs act could lead to new approaches for therapy in diverse renal diseases...
  11. pmc Fenofibric acid reduces fibronectin and collagen type IV overexpression in human retinal pigment epithelial cells grown in conditions mimicking the diabetic milieu: functional implications in retinal permeability
    Kyle Trudeau
    Department of Medicine, Boston University School of Medicine, Boston, Massachusetts 02118, USA
    Invest Ophthalmol Vis Sci 52:6348-54. 2011
    ..To determine whether fenofibric acid (FA) reduces high glucose (HG)-induced basement membrane component overexpression and hyperpermeability in human retinal pigment epithelial (RPE) cells...
  12. ncbi Alport's syndrome, Goodpasture's syndrome, and type IV collagen
    Billy G Hudson
    Department of Medicine, Vanderbilt University School of Medicine, Nashville, TN 37232 2358, USA
    N Engl J Med 348:2543-56. 2003
  13. ncbi Basement membranes and human disease
    Tom Van Agtmael
    University of Glasgow, UK
    Cell Tissue Res 339:167-88. 2010
    ..Despite this, exciting progress has recently been made with potential treatment options for some of these so far incurable diseases...
  14. ncbi Role of COL4A1 in small-vessel disease and hemorrhagic stroke
    Douglas B Gould
    Howard Hughes Medical Institute, Bar Harbor, ME, USA
    N Engl J Med 354:1489-96. 2006
    ..We concluded that mutation of COL4A1 may cause a spectrum of cerebrovascular phenotypes and that persons with COL4A1 mutations may be predisposed to hemorrhage, especially after environmental stress...
  15. pmc Basement membrane components are key players in specialized extracellular matrices
    Jenny Kruegel
    Tissue Regeneration Work Group, Department of Prosthodontics, Georg August University, Robert Koch Str 40, 37075, Gottingen, Germany
    Cell Mol Life Sci 67:2879-95. 2010
    ..Here, we briefly review the latest research on these unique sECM and their components with a special emphasis on embryonic and adult stem cells and their niches...
  16. pmc COL4A2 mutations impair COL4A1 and COL4A2 secretion and cause hemorrhagic stroke
    Marion Jeanne
    Departments of Ophthalmology and Anatomy, Institute for Human Genetics, University of California, San Francisco School of Medicine, 94143, USA
    Am J Hum Genet 90:91-101. 2012
    ..Our data suggest that COL4A2 mutations impair COL4A1 and COL4A2 secretion and can also result in cytotoxicity. Finally, our findings suggest that, collectively, mutations in COL4A1 and COL4A2 contribute to sporadic cases of ICH...
  17. pmc The epidermal basement membrane is a composite of separate laminin- or collagen IV-containing networks connected by aggregated perlecan, but not by nidogens
    Daniel Timo Behrens
    Institute for Physiological Chemistry and Pathobiochemistry, University Hospital of Munster, Munster, Germany
    J Biol Chem 287:18700-9. 2012
    ..We conclude that the epidermal basement membrane is a composite of two structurally independent networks that are tightly connected in a spot-welding-like manner by perlecan-containing aggregates...
  18. pmc Drosophila laminins act as key regulators of basement membrane assembly and morphogenesis
    Jose M Urbano
    Centro Andaluz de Biología de Desarrollo CABD, Univ Pablo de Olavide CSIC, 41013 Sevilla, Spain
    Development 136:4165-76. 2009
    ..We propose that while an early function of laminins in gastrulation is not conserved in Drosophila and mammals, their function in basement membrane assembly and organogenesis seems to be maintained throughout evolution...
  19. ncbi Col4a1 mutation causes endoplasmic reticulum stress and genetically modifiable ocular dysgenesis
    Douglas B Gould
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    Hum Mol Genet 16:798-807. 2007
    ..In a different genetic context, both the ASD and optic nerve hypoplasia are rescued, and we have identified a single dominant locus that confers the phenotypic modification...
  20. ncbi X-linked Alport syndrome: natural history and genotype-phenotype correlations in girls and women belonging to 195 families: a "European Community Alport Syndrome Concerted Action" study
    Jean Philippe Jais
    Biostatistique et Informatique Médicale, Hopital Necker Enfants Malades, Universite Rene Descartes, Paris, France
    J Am Soc Nephrol 14:2603-10. 2003
    ..Risk factors for developing renal failure have been identified: the occurrence and progressive increase in proteinuria, and the development of a hearing defect...
  21. pmc Assembly of lamina-specific neuronal connections by slit bound to type IV collagen
    Tong Xiao
    Programs in Neuroscience, Department of Physiology, University of California, San Francisco, 1550 Fourth Street, San Francisco, CA 94158 2722, USA
    Cell 146:164-76. 2011
    ..We propose that Slit1 signaling, perhaps in the form of a superficial-to-deep gradient, presents laminar positional cues to ingrowing retinal axons...
  22. ncbi Role of laminin terminal globular domains in basement membrane assembly
    Karen K McKee
    Department of Pathology and Laboratory Medicine, Robert Wood Johnson Medical School, Piscataway, New Jersey 08854, USA
    J Biol Chem 282:21437-47. 2007
    ..Furthermore, type IV collagen recruitment into the laminin extracellular matrices appears to be mediated through a nidogen bridge with a lesser contribution arising from a direct interaction with laminin...
  23. pmc Advances in Alport syndrome diagnosis using next-generation sequencing
    Rosangela Artuso
    Medical Genetics Section, Biotechnology Department, University of Siena, Siena, Italy
    Eur J Hum Genet 20:50-7. 2012
    ..Arg1682Trp in COL4A4) and to reconsider the diagnosis of ATS in a third patient. This study, therefore, illustrates the successful application of NGS to mutation screening of Mendelian disorders with locus heterogeneity...
  24. ncbi Dominant mutations of Col4a1 result in basement membrane defects which lead to anterior segment dysgenesis and glomerulopathy
    Tom Van Agtmael
    Molecular Physiology, Centre for Cardiovascular Science, University of Edinburgh, UK
    Hum Mol Genet 14:3161-8. 2005
    ....
  25. ncbi Reduction of tumstatin in asthmatic airways contributes to angiogenesis, inflammation, and hyperresponsiveness
    Janette K Burgess
    Respiratory Research Group, Discipline of Pharmacology, Bosch Building, D05, University of Sydney, Sydney, NSW, Australia 2006
    Am J Respir Crit Care Med 181:106-15. 2010
    ..Collagen IV is decreased in the airway basement membrane in asthma. It has six alpha chains, of which the noncollagenous domain-1 domains have endogenous antiangiogenic properties...
  26. ncbi Rescue of mesangial cells from high glucose-induced over-proliferation and extracellular matrix secretion by hydrogen sulfide
    Ping Yuan
    Department of Physiology and Pathophysiology, Shanghai Medical College, Fudan University Shanghai 200032, China
    Nephrol Dial Transplant 26:2119-26. 2011
    ..The present study aimed to explore the changes in H(2)S metabolism in the early stage of DN and the effects of H(2)S on cultured rat renal glomerular mesangial cells (MCs)...
  27. ncbi High ambient glucose levels modulates the production of MMP-9 and alpha5(IV) collagen by cultured podocytes
    Yaling Bai
    Division of Nephrology, Peking University First Hospital, Beijing, China
    Cell Physiol Biochem 17:57-68. 2006
    ....
  28. pmc Characterization of the anti-angiogenic properties of arresten, an alpha1beta1 integrin-dependent collagen-derived tumor suppressor
    Pia Nyberg
    Center for Matrix Biology, Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA, USA
    Exp Cell Res 314:3292-305. 2008
    ..Collectively, this data sheds more light into the anti-angiogenic mechanism of arresten...
  29. ncbi Severe Alport syndrome in a young woman caused by a t(X;1)(q22.3;p36.32) balanced translocation
    Kazumoto Iijima
    Department of Pediatrics, Division of Child Health and Development, Kobe University Graduate School of Medicine, 7 5 2 Kusunoki cho, Chuo Ku, Kobe, 650 0017, Japan
    Pediatr Nephrol 25:2165-70. 2010
    ..G-banding chromosomal analysis showed a t(X;1)(q22.3;p36.32) balanced translocation. Analysis of the collagen type IV (COL4A5) gene by genomic DNA sequencing, complementary DNA (cDNA) sequencing and multiplex ligation-dependent ..
  30. ncbi COL4A3/COL4A4 mutations producing focal segmental glomerulosclerosis and renal failure in thin basement membrane nephropathy
    Konstantinos Voskarides
    Department of Biological Sciences, University of Cyprus, Nicosia, Cyprus
    J Am Soc Nephrol 18:3004-16. 2007
    ..The findings presented here do not justify the labelling of thin basement membrane nephropathy as a benign condition with excellent prognosis...
  31. ncbi Autosomal-dominant Alport syndrome: natural history of a disease due to COL4A3 or COL4A4 gene
    Chiara Pescucci
    Medical Genetics, Department of Molecular Biology, University of Siena, Siena, Italy
    Kidney Int 65:1598-603. 2004
    ..Because of the paucity of the reported families, the natural history of autosomal-dominant Alport syndrome is mostly unknown...
  32. ncbi p53 inhibits angiogenesis by inducing the production of Arresten
    Sarah Assadian
    Goodman Cancer Research Center, Department of Biochemistry, McGill University, Montreal, Quebec, Canada
    Cancer Res 72:1270-9. 2012
    ..Our findings, therefore, link the production of Arresten to the p53 tumor suppressor pathway and show a novel mechanism through which p53 can inhibit angiogenesis...
  33. ncbi Expression of type IV collagen and matrix metalloproteinase-2 (type IV collagenase) in relation to nodal status in laryngeal cancer
    T Krecicki
    Department of Otolaryngology, Medical University of Wrocław, Poland
    Clin Otolaryngol Allied Sci 26:469-72. 2001
    ....
  34. ncbi Herpes-simplex virus encephalitis is characterized by an early MMP-9 increase and collagen type IV degradation
    Johann Sellner
    Department of Neurology, University Hospital Bern, Inselspital, Freiburgstrasse, CH 3010 Bern, Switzerland
    Brain Res 1125:155-62. 2006
    ..We examined changes of collagen type IV, the major constituent of the neurovascular matrix, together with expression and localization of matrix-..
  35. pmc Venular basement membranes ubiquitously express matrix protein low-expression regions: characterization in multiple tissues and remodeling during inflammation
    Mathieu Benoit Voisin
    Barts and the London School of Medicine and Dentistry, Queen Mary University of London, William Harvey Research Institute, London EC1M 6BQ, UK
    Am J Pathol 176:482-95. 2010
    ..Collectively the findings provide further insight into characteristics of specialized regions within venular basement membranes that are preferentially used and remodeled by transmigrating neutrophils...
  36. ncbi Enrichment of putative human epidermal stem cells based on cell size and collagen type IV adhesiveness
    Juxue Li
    State Key Laboratory of Reproductive Biology, Institute of Zoology, Chinese Academy of Sciences, Datun Road, Chaoyang District, Beijing 100101, China
    Cell Res 18:360-71. 2008
    ..Here we report that cell size combined with collagen type IV adhesiveness can be used in an improved approach to enrich pure and viable human EpSCs...
  37. pmc Type IV procollagen missense mutations associated with defects of the eye, vascular stability, the brain, kidney function and embryonic or postnatal viability in the mouse, Mus musculus: an extension of the Col4a1 allelic series and the identification of
    Jack Favor
    Institute of Human Genetics, GSF National Research Center for Environment and Health, D 85764 Neuherberg, Germany
    Genetics 175:725-36. 2007
    ..We suggest that the spontaneous intraorbital hemorrhages observed in the mouse are a clinically relevant phenotype with a relatively high predictive value to identify carriers of COL4A1 or COL4A2 mutations...
  38. ncbi Cathepsin S controls angiogenesis and tumor growth via matrix-derived angiogenic factors
    Bing Wang
    Cardiovascular Medicine, Department of Medicine, Brigham and Women s Hospital and Harvard Medical School, 77 Ave Louis Pasteur, Boston, MA 02115, USA
    J Biol Chem 281:6020-9. 2006
    ....
  39. pmc Lack of integrin alpha1beta1 leads to severe glomerulosclerosis after glomerular injury
    Xiwu Chen
    Department of Medicine, Division of Nephrology and Hypertension, Vanderbilt University, Medical Center North, B3109, 1161 21st Ave, Nashville, TN 37212, USA
    Am J Pathol 165:617-30. 2004
    ..Because integrin alpha1beta1 is expressed in many cell types this may represent a generalized mechanism of controlling matrix accumulation, which has implications for numerous diseases characterized by fibrosis...
  40. ncbi Identification of the anti-angiogenic site within vascular basement membrane-derived tumstatin
    Y Maeshima
    Department of Medicine and the Cancer Center, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts 02215, USA
    J Biol Chem 276:15240-8. 2001
    ..Taken together, these findings demonstrate that Tum-5, a domain derived from tumstatin, is an effective inhibitor of tumor-associated angiogenesis and a promising candidate for the treatment of cancer...
  41. ncbi Pericardin, a Drosophila type IV collagen-like protein is involved in the morphogenesis and maintenance of the heart epithelium during dorsal ectoderm closure
    Aymeric Chartier
    Laboratoire de Genetique et Physiologie du Developpement, UMR 6545 CNRS Université, IBDM CNRS INSERM Université de la Méditerranée, Campus de Luminy, Case 907, 13288 Marseille Cedex 09, France
    Development 129:3241-53. 2002
    ..Our results support, for the first time, the function of a collagen-like protein in the coordinated migration of dorsal ectoderm and heart cells...
  42. ncbi Integrated actions of transforming growth factor-beta1 and connective tissue growth factor in renal fibrosis
    W Qi
    Dept of Medicine, Level 3, Wallace Freeborn Professorial Block, Royal North Shore Hospital, St Leonards, New South Wales, Australia 2065, USA
    Am J Physiol Renal Physiol 288:F800-9. 2005
    ..It has further uniquely demonstrated that CTGF requires TGF-beta, signaling through the TbetaRII in both PTCs and CFs, to exert its fibrogenic response in this in vitro model...
  43. ncbi Alpha(v)beta3 and alpha(v)beta5 integrins bind both the proximal RGD site and non-RGD motifs within noncollagenous (NC1) domain of the alpha3 chain of type IV collagen: implication for the mechanism of endothelia cell adhesion
    Vadim Pedchenko
    Division of Nephrology, Veterans Affairs Hospital, Vanderbilt University Medical Center, Nashville, Tennessee 37232, USA
    J Biol Chem 279:2772-80. 2004
    ..Therefore, the RGD site, in addition to non-RGD motifs, may contribute to the mechanisms of endothelial cell adhesion in the human vasculature and the anti-angiogenic activity of the RGD-alpha3NC1 domain...
  44. ncbi COL4A3/COL4A4 mutations: from familial hematuria to autosomal-dominant or recessive Alport syndrome
    Ilaria Longo
    Genetica Medica, Universita di Siena, Siena Dip Scienze Cliniche e Biologiche and Genetica, Biologia e Biochimica, Universita di Torino, Torino, Italy
    Kidney Int 61:1947-56. 2002
    ..COL4A3/COL4A4 mutations: From familial hematuria to autosomal-dominant or recessive Alport syndrome...
  45. ncbi Caveolin-1 mediates the expression and localization of cathepsin B, pro-urokinase plasminogen activator and their cell-surface receptors in human colorectal carcinoma cells
    Dora Cavallo-Medved
    Department of Pharmacology, Wayne State University, School of Medicine, Detroit, MI 48201, USA
    J Cell Sci 118:1493-503. 2005
    ....
  46. pmc Role of the podocyte (and glomerular endothelium) in building the GBM
    Dale R Abrahamson
    Department of Anatomy and Cell Biology, University of Kansas Medical Center, Kansas City, KS 66160, USA
    Semin Nephrol 32:342-9. 2012
    ..Stress is placed on the assembly of a compositionally correct GBM for the acquisition and maintenance of glomerular barrier properties...
  47. ncbi Essential environmental cues from the satellite cell niche: optimizing proliferation and differentiation
    K J M Boonen
    Department of Biomedical Engineering, Eindhoven University of Technology, Eindhoven, The Netherlands
    Am J Physiol Cell Physiol 296:C1338-45. 2009
    ..However, ongoing maturation with cross-striations and contractions occurred only on elasticities higher than 3 kPa. Furthermore, maturation was fastest on poly-d-lysine and laminin coatings...
  48. ncbi Type IV collagen and CD44v6 expression in benign, malignant primary and metastatic ovarian tumors: correlation with Ki-67 and p53 immunoreactivity
    Julia K Bar
    Department of Clinical Immunology, Medical University, 50 368 Wrocław, Poland
    Gynecol Oncol 95:23-31. 2004
    ..This study investigates the expression of type IV collagen, CD44v6 molecule in correlation with p53 and Ki-67 presence in primary and metastatic lesion of ovarian carcinoma to define their role in metastases of ovarian carcinoma...
  49. pmc COL4A1 mutations in patients with sporadic late-onset intracerebral hemorrhage
    Yi Chinn Weng
    Department of Ophthalmology, Institute for Human Genetics, UCSF School of Medicine, San Francisco, CA, USA
    Ann Neurol 71:470-7. 2012
    ..Mutations in the type IV collagen alpha 1 gene (COL4A1) cause dominantly inherited cerebrovascular disease. We seek to determine the extent to which COL4A1 mutations contribute to sporadic, nonfamilial, intracerebral hemorrhages (ICHs)...
  50. pmc Human alpha1 type IV collagen NC1 domain exhibits distinct antiangiogenic activity mediated by alpha1beta1 integrin
    Akulapalli Sudhakar
    Cell Signaling and Angiogenesis Laboratory, Department of Genetics, Boys Town National Research Hospital, Omaha, Nebraska 68131, USA
    J Clin Invest 115:2801-10. 2005
    ..Collectively, the above data indicate that alpha1(IV)NC1 is a potential therapeutic candidate for targeting tumor angiogenesis...
  51. ncbi Angiotensin II activation of the JAK/STAT pathway in mesangial cells is altered by high glucose
    Farhad Amiri
    Vascular Biology Center, Department of Pharmacology and Toxicology, Medical College of Georgia, Augusta, Georgia 30912 2500, USA
    Kidney Int 61:1605-16. 2002
    ..We hypothesized that Ang II activation of the JAK/STAT pathway is altered by HG in GMC, and that this pathway might be linked to the Ang II-induced growth and overproduction of collagen IV in GMC in HG conditions...
  52. pmc Partial enrichment of a population of human limbal epithelial cells with putative stem cell properties based on collagen type IV adhesiveness
    De Quan Li
    Ocular Surface Center, Cullen Eye Institute, Department of Ophthalmology, Baylor College of Medicine, 6565 Fannin Street, NC 205, Houston, TX 77030, USA
    Exp Eye Res 80:581-90. 2005
    ..The RAC population enriched for certain putative stem cell properties may prove useful in the future for transplantation to diseased and damaged corneas with limbal stem cell deficiency...
  53. pmc Recruitment of focal adhesion kinase and paxillin to beta1 integrin promotes cancer cell migration via mitogen activated protein kinase activation
    David L Crowe
    Center for Craniofacial Molecular Biology, University of Southern California, 2250 Alcazar Street, Los Angeles, CA 90033, USA
    BMC Cancer 4:18. 2004
    ..We previously demonstrated that MAPK activation was required for growth factor induced in vitro migration and invasion by human squamous cell carcinoma (SCC) lines...
  54. pmc Tetrastatin, the NC1 domain of the α4(IV) collagen chain: a novel potent anti-tumor matrikine
    Sylvie Brassart-Pasco
    CNRS UMR 6237, Universite de Reims Champagne Ardenne, Reims, France
    PLoS ONE 7:e29587. 2012
    ..NC1 domains from α1, α2, α3 and α6(IV) collagen chains were shown to exert anti-tumor or anti-angiogenic activities, whereas the NC1 domain of the α4(IV) chain did not show such activities so far...
  55. ncbi Stem cell-derived Sca-1+ progenitors differentiate into smooth muscle cells, which is mediated by collagen IV-integrin alpha1/beta1/alphav and PDGF receptor pathways
    Qingzhong Xiao
    Department of Cardiac and Vascular Sciences, St George s, University of London, Cranmer Terrace, London SW17 0RE, UK
    Am J Physiol Cell Physiol 292:C342-52. 2007
    ....
  56. pmc Cellular origins of type IV collagen networks in developing glomeruli
    Dale R Abrahamson
    Department of Anatomy and Cell Biology, The Kidney Institute, University of Kansas Medical Center, 3901 Rainbow Boulevard, MS 3038, Kansas City, KS 66160, USA
    J Am Soc Nephrol 20:1471-9. 2009
    ..Together, these results indicate that collagen alpha 3 alpha 4 alpha 5(IV) originates solely from podocytes; therefore, glomerular Alport disease is a genetic defect that manifests specifically within this cell type...
  57. pmc COL4A1 is associated with arterial stiffness by genome-wide association scan
    Kirill V Tarasov
    Laboratory of Cardiovascular Science, Laboratory of Genetics, Clinical Research Branch, Intramural Research Program, National Institute on Aging, National Institutes of Health, Baltimore, MD, USA
    Circ Cardiovasc Genet 2:151-8. 2009
    ..Heritability and linkage studies have pointed toward a genetic component affecting PWV. We conducted a genome-wide association study to identify single-nucleotide polymorphisms (SNPs) associated with PWV...
  58. ncbi Clinical spectrum of type IV collagen (COL4A1) mutations: a novel genetic multisystem disease
    Katayoun Vahedi
    Department of Neurology, Lariboisiere Hospital, France
    Curr Opin Neurol 24:63-8. 2011
    ..COL4A1 encodes type IV collagen α1 chain, a crucial component of nearly all basement membrane including vasculature, renal glomerule and ocular structures...
  59. ncbi Immunohistochemical assessment of basement membrane components in colorectal cancer: prognostic implications
    A Ch Lazaris
    Department of Pathology, School of Medicine, The National and Capodistrian University of Athens, Athens, Greece
    J Exp Clin Cancer Res 22:599-606. 2003
    ..The total loss or considerable discontinuity of the basement membranes of cancerous cells can be considered as indicators of tumour aggressiveness...
  60. pmc COL4A2 mutation associated with familial porencephaly and small-vessel disease
    Elly Verbeek
    Department of Clinical Genetics, Erasmus University Medical Center, Rotterdam, The Netherlands
    Eur J Hum Genet 20:844-51. 2012
    ....
  61. ncbi Expression, purification, and bioactivity of human tumstatin from Escherichia coli
    Quliang Gu
    The Key Laboratory of Gene Engineering of Ministry of Education, Sun Yat Sen University, Guangzhou, China
    Protein Expr Purif 47:461-6. 2006
    ....
  62. pmc De novo and inherited mutations in COL4A2, encoding the type IV collagen α2 chain cause porencephaly
    Yuriko Yoneda
    Department of Human Genetics, Yokohama City University Graduate School of Medicine, Fukuura, Kanazawa Ku, Japan
    Am J Hum Genet 90:86-90. 2012
    ..The c.3110G>A mutation occurred de novo. Our study confirmed that abnormalities of the α1α1α2 heterotrimers of type IV collagen cause porencephaly and stresses the importance of screening for COL4A2 as well as for COL4A1...
  63. ncbi Improvement of the dermal epidermal junction in human reconstructed skin by a new c-xylopyranoside derivative
    Juliette Sok
    L Oreal Recherche, Centre de Recherche C Zviak, 90 rue du General Roguet, 92583 Clichy Cedex, France
    Eur J Dermatol 18:297-302. 2008
    ....
  64. pmc Counterbalancing angiogenic regulatory factors control the rate of cancer progression and survival in a stage-specific manner
    Liang Xie
    Division of Matrix Biology, Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02215, USA
    Proc Natl Acad Sci U S A 108:9939-44. 2011
    ..The results demonstrate that EAIs are part of a balance mechanism regulating tumor angiogenesis, serving as intrinsic microenvironmental barriers to tumorigenesis...
  65. pmc The glomerular basement membrane
    Jeffrey H Miner
    Renal Division and Dept of Cell Biology and Physiology, Washington University School of Medicine, St Louis, MO 63110, USA
    Exp Cell Res 318:973-8. 2012
    ..These studies suggest that the GBM plays a crucial role in establishing and maintaining the glomerular filtration barrier...
  66. ncbi Basement membrane reconstruction in human skin equivalents is regulated by fibroblasts and/or exogenously activated keratinocytes
    Abdoelwaheb El Ghalbzouri
    Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands
    J Invest Dermatol 124:79-86. 2005
    ....
  67. ncbi Basement membrane proteins play an important role in the invasive processes of human pancreatic cancer cells
    Hirozumi Sawai
    Department of Gastroenterological Surgery, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
    J Surg Res 144:117-23. 2008
    ..Furthermore, we examined the roles of beta(1)-integrins and arginine-glycine-aspartic (RGD)-containing oligopeptide in cell-matrix interactions...
  68. ncbi Accumulation of type IV collagen in dilated ER leads to apoptosis in Hsp47-knockout mouse embryos via induction of CHOP
    Toshihiro Marutani
    Department of Molecular and Cellular Biology and CREST JST, Institute for Frontier Medical Sciences, Kyoto University, Sakyo ku, Kyoto 606 8397, Japan
    J Cell Sci 117:5913-22. 2004
    ..5 dpc together with significantly upregulated CHOP mRNA expression. ER stress caused by the accumulation of misfolded collagen may have induced apoptosis in Hsp47-knockout embryos through the upregulation of CHOP...
  69. ncbi Type IV collagen alpha6 chain-derived noncollagenous domain 1 (alpha6(IV)NC1) inhibits angiogenesis and tumor growth
    Thomas M Mundel
    Division of Matrix Biology, Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts 02215, USA
    Int J Cancer 122:1738-44. 2008
    ..Inhibition of tumor growth is associated with significantly diminished microvascular density. Collectively, our results demonstrate that alpha6NC1 is an endogenous inhibitor of angiogenesis and tumor growth...
  70. ncbi Novel COL4A1 mutations associated with HANAC syndrome: a role for the triple helical CB3[IV] domain
    Emmanuelle Plaisier
    AP HP, Department of Nephrology, Tenon Hospital, INSERM U702, UMRS702, Universite Pierre et Marie Curie Paris 6, Paris, France
    Am J Med Genet A 152:2550-5. 2010
    ....
  71. ncbi Age-dependent changes in the structure, composition and biophysical properties of a human basement membrane
    Joseph Candiello
    Department of Bioengineering, University of Pittsburgh, North Carolina Central University, United States
    Matrix Biol 29:402-10. 2010
    ..Force-indentation measurements by AFM also showed that ILMs become increasingly stiffer with advancing age. The data suggest that BMs from other human tissues may undergo similar age-related changes...
  72. ncbi Molecular interactions in the retinal basement membrane system: a proteomic approach
    Manimalha Balasubramani
    Proteomics Core, Genomics and Proteomics Core Laboratories, University of Pittsburgh, Pittsburgh, PA 15261, USA
    Matrix Biol 29:471-83. 2010
    ..To our knowledge, this is the first large-scale investigation of an in vivo BM system using MS-based proteomics...
  73. ncbi The YSNSG cyclopeptide derived from tumstatin inhibits tumor angiogenesis by down-regulating endothelial cell migration
    Jessica Thevenard
    CNRS UMR 6237, Universite de Reims Champagne Ardenne, CHU de Reims, Reims, France
    Int J Cancer 126:1055-66. 2010
    ..Our results confirm the YSNSG cyclopeptide as a potent antitumor agent, through both the inhibition of invasive properties of tumor cells and the antiangiogenic activity...
  74. ncbi Effects of benazepril on renal function and kidney expression of matrix metalloproteinase-2 and tissue inhibitor of metalloproteinase-2 in diabetic rats
    Shu Zhen Sun
    Department of Pediatrics, Shandong Provincial Hospital, Shandong University, Jinan 250021, China
    Chin Med J (Engl) 119:814-21. 2006
    ....
  75. ncbi Spontaneous regeneration of the corticospinal tract after transection in young rats: collagen type IV deposition and astrocytic scar in the lesion site are not the cause but the effect of failure of regeneration
    Tsutomu Iseda
    Department of Integrative Brain Science, Graduate School of Medicine, Kyoto University, Kyoto 606 8501, Japan
    J Comp Neurol 464:343-55. 2003
    ..To identify cells and associated molecules that promote or impede regeneration, we compared expression of collagen type IV, glial fibrillary acidic protein (GFAP), and vimentin immunoreactivity (IR) at the lesion sites in ..
  76. pmc Abnormal expression of collagen IV in lens activates unfolded protein response resulting in cataract
    Zeynep Firtina
    Department of Biological Sciences, University of Delaware, Newark, Delaware 19716, USA
    J Biol Chem 284:35872-84. 2009
    ..Collectively, the results provide in vivo evidence for a role of UPR in cataract formation in response to accumulation of terminally unfolded proteins in the endoplasmic reticulum...
  77. ncbi Glomerular injury is exacerbated in diabetic integrin alpha1-null mice
    R Zent
    Department of Medicine, Division of Nephrology, Medical Center North, Vanderbilt University, Nashville, Tennessee 37232, USA
    Kidney Int 70:460-70. 2006
    ..In conclusion, this study suggests that lack of integrin alpha1beta1 exacerbates the glomerular injury in a mouse model of diabetes by modulating GFR, ROS production, cell proliferation, and collagen deposition...
  78. pmc Retinal basement membrane abnormalities and the retinopathy of Alport syndrome
    Judy Savige
    Department of Medicine Northern Health, University of Melbourne, Melbourne, Vic, Australia
    Invest Ophthalmol Vis Sci 51:1621-7. 2010
    ..To determine the effects of X-linked and autosomal recessive Alport syndrome on retinal basement membranes and how these result in the characteristic perimacular dot-and-fleck retinopathy, lozenge, and macular hole...
  79. ncbi Laminin compensation in collagen alpha3(IV) knockout (Alport) glomeruli contributes to permeability defects
    Dale R Abrahamson
    Department of Anatomy and Cell Biology, University of Kansas Medical Center, MS 3038, 3901 Rainbow Boulevard, Kansas City, KS 66160, USA
    J Am Soc Nephrol 18:2465-72. 2007
    ....
  80. pmc FAK and p38-MAP kinase-dependent activation of apoptosis and caspase-3 in retinal endothelial cells by alpha1(IV)NC1
    Chandra S Boosani
    Cell Signaling and Tumor Angiogenesis Laboratory, Department of Genetics, Boys Town National Research Hospital, Omaha, Nebraska 68131, USA
    Invest Ophthalmol Vis Sci 50:4567-75. 2009
    ..To determine the impact of the antiangiogenic factor alpha1(IV)NC1 on vascular endothelial growth factor-mediated proangiogenic activity in mouse retinal endothelial cells (MRECs)...
  81. ncbi The antitumor properties of the alpha3(IV)-(185-203) peptide from the NC1 domain of type IV collagen (tumstatin) are conformation-dependent
    Nicolas Floquet
    Laboratory of Spectroscopy and BioMolecular Structures, EA 3305, CNRS Unite Mixte de Recherche 6013, Institut Federatif de Recherche 53 Biomolecules, University of Reims Champagne Ardenne, Reims F 51095, France
    J Biol Chem 279:2091-100. 2004
    ..These peptides could act as potent and specific antitumor antagonists of alpha(v)beta(3) integrin in melanoma progression...
  82. ncbi Mutations in the COL4A4 gene in thin basement membrane disease
    Mark Buzza
    University of Melbourne, Department of Medicine, Austin and Repatriation Medical Centre, Heidelberg, Australia
    Kidney Int 63:447-53. 2003
    ..These genes also are affected in autosomal recessive Alport syndrome. The aim of this study was to demonstrate COL4A4 mutations in TBMD...
  83. ncbi Novel COL4A4 splice defect and in-frame deletion in a large consanguine family as a genetic link between benign familial haematuria and autosomal Alport syndrome
    Oliver Gross
    Medical Faculty University of Cologne, Cologne General Hospital, Department of Internal Medicine I, Merheim Medical Center, Cologne, Germany
    Nephrol Dial Transplant 18:1122-7. 2003
    ..The present study describes molecular changes of the COL4A4 gene that cause both diseases: autosomal recessive AS and BFH in a consanguine family with a 400-year-old history of haematuria...
  84. pmc Polymorphisms in COL4A3 and COL4A4 genes associated with keratoconus
    Mirna Stabuc-Silih
    Eye Hospital, University Medical Centre, Ljubljana, Slovenia
    Mol Vis 15:2848-60. 2009
    Alterations in collagen type IV, alpha-3 (COL4A3) and collagen type IV, alpha-4 (COL4A4) genes may be responsible for a decrease in collagen types I and III, a feature often detected in keratoconus (KC)...
  85. pmc COL4A1 mutation in preterm intraventricular hemorrhage
    Kaya Bilguvar
    Department of Neurosurgery, Yale University School of Medicine, New Haven, CT 06520, USA
    J Pediatr 155:743-5. 2009
    ..We report a rare variant in COL4A1 associated with intraventricular hemorrhage in dizygotic preterm twins. These results expand the spectrum of diseases attributable to mutations in type IV procollagens...
  86. pmc Novel mutations in three families confirm a major role of COL4A1 in hereditary porencephaly
    G Breedveld
    Department of Clinical Genetics, Erasmus University Medical Centre, PO Box 1738, 3000 DR Rotterdam, Netherlands
    J Med Genet 43:490-5. 2006
    ..A report of inherited mutations in COL4A1 in two families has shown that familial porencephaly may have the same cause in humans...
  87. ncbi COL4A1 mutation in two preterm siblings with antenatal onset of parenchymal hemorrhage
    Linda S de Vries
    Department of Neonatology, Wilhelmina Children s Hospital, UMC Utrecht, The Netherlands
    Ann Neurol 65:12-8. 2009
    ..To report the presence of intracerebral hemorrhage and porencephaly, both present at birth, in two preterm infants with a mutation in the collagen 4 A1 gene...
  88. pmc A human-mouse chimera of the alpha3alpha4alpha5(IV) collagen protomer rescues the renal phenotype in Col4a3-/- Alport mice
    Laurence Heidet
    Inserm U574, Hopital Necker Enfants Malades, Universite Rene Descartes, Paris, France
    Am J Pathol 163:1633-44. 2003
    ..This line of mice, humanized for the alpha3(IV) collagen chain, will also provide a valuable model for studying the pathogenesis of Goodpasture syndrome, an autoimmune disease caused by antibodies against this chain...
  89. ncbi COL4A1 mutation in Axenfeld-Rieger anomaly with leukoencephalopathy and stroke
    Igor Sibon
    Centre Hospitalier Universitaire Bordeaux, Fédération des Neurosciences Cliniques, Hopital Pellegrin, Bordeaux, France
    Ann Neurol 62:177-84. 2007
    ..Some of them have ophthalmological, mainly retinal, manifestations. Herein, we report on a family affected by vascular leukoencephalopathy and variable abnormalities of the anterior chamber of the eye...
  90. ncbi Ocular gene transfer of active TGF-beta induces changes in anterior segment morphology and elevated IOP in rats
    Jennifer V Robertson
    Department of Pathology and Molecular Medicine, McMaster University, Hamilton, Ontario, Canada
    Invest Ophthalmol Vis Sci 51:308-18. 2010
    ..Gene transfer of TGF-beta can, therefore, be used to induce anatomic changes in the anterior segment in a rodent model that result in ocular hypertension...
  91. ncbi Regulation of human beta-cell adhesion, motility, and insulin secretion by collagen IV and its receptor alpha1beta1
    Thomas Kaido
    Department of Pediatrics, Islet Research Laboratory at The Whittier Institute for Diabetes, University of California at San Diego, La Jolla, CA 92037, USA
    J Biol Chem 279:53762-9. 2004
    ..Cultured beta-cells adhered to and migrated on collagen type IV (Col-IV), and these responses were mediated almost exclusively by alpha(1)beta(1)...
  92. pmc Stage-specific action of matrix metalloproteinases influences progressive hereditary kidney disease
    Michael Zeisberg
    Center for Matrix Biology, Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts, USA
    PLoS Med 3:e100. 2006
    ..The pathological mechanisms by which such mutations lead to eventual kidney failure are not completely understood...
  93. ncbi Collagen XVII participates in keratinocyte adhesion to collagen IV, and in p38MAPK-dependent migration and cell signaling
    Hongjiang Qiao
    Creative Research Initiative, Hokkaido University, Sapporo, Japan
    J Invest Dermatol 129:2288-95. 2009
    ....
  94. ncbi The molecular basis of Goodpasture and Alport syndromes: beacons for the discovery of the collagen IV family
    Billy G Hudson
    Department of Medicine, Vanderbilt University School of Medicine, B 3102 Medical Center North, 1161 21st Avenue South, Nashville, TN 37232 2372, USA
    J Am Soc Nephrol 15:2514-27. 2004
  95. ncbi Sixteen novel mutations identified in COL4A3, COL4A4, and COL4A5 genes in Slovenian families with Alport syndrome and benign familial hematuria
    M Slajpah
    Department of Molecular Genetics, Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
    Kidney Int 71:1287-95. 2007
    ..Sixteen mutations are to the best of our knowledge new and private...
  96. ncbi Type IV collagens regulate BMP signalling in Drosophila
    Xiaomeng Wang
    Faculty of Life Sciences, The University of Manchester, Oxford Road, Manchester M13 9PT, UK
    Nature 455:72-7. 2008
    ..On the basis of our findings that human type IV collagen binds BMP4, we predict that this role of type IV collagens will be conserved...
  97. ncbi Angiotensin II stimulates alpha3(IV) collagen production in mouse podocytes via TGF-beta and VEGF signalling: implications for diabetic glomerulopathy
    Sheldon Chen
    Renal Electrolyte and Hypertension Division of the University of Pennsylvania, Philadelphia, PA, USA
    Nephrol Dial Transplant 20:1320-8. 2005
    ..Podocyte expression of alpha3(IV) collagen may involve the transforming growth factor-beta (TGF-beta) and vascular endothelial growth factor (VEGF) systems...
  98. ncbi Structure and function of basement membranes
    Valerie S LeBleu
    Division of Matrix Biology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts 02215, USA
    Exp Biol Med (Maywood) 232:1121-9. 2007
    ..Additionally, the resulting endothelial and epithelial heterogeneity in human body is a direct contribution of cell-matrix interaction facilitated by the diverse compositions of BMs...
  99. pmc Rheumatic fever-associated Streptococcus pyogenes isolates aggregate collagen
    Katrin Dinkla
    Department of Microbial Pathogenesis and Vaccine Research, Gesellschaft für Biotechnologische Forschung German Research Centre for Biotechnology, Braunschweig, Germany
    J Clin Invest 111:1905-12. 2003
    ..during outbreaks of rheumatic fever have the unique capability to bind and aggregate human basement membrane collagen type IV. M3 protein is identified as collagen-binding factor of M3 streptococci, whereas M18 isolates bind collagen ..
  100. ncbi Thiazolidinediones ameliorate diabetic nephropathy via cell cycle-dependent mechanisms
    Tatsuo Okada
    Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, 2 5 1 Shikata cho, Okayama 700 8558, Japan
    Diabetes 55:1666-77. 2006
    ..Besides glucose-lowering action, pioglitazone ameliorates diabetic nephropathy via cell cycle-dependent mechanisms...
  101. ncbi Haemocyte-derived SPARC is required for collagen-IV-dependent stability of basal laminae in Drosophila embryos
    Nathalie Martinek
    Department of Cell and Systems Biology, University of Toronto, 25 Harbord Street, Toronto, Ontario, M5S 3G5, Canada
    J Cell Sci 121:1671-80. 2008
    ..Hence, SPARC is required for basal lamina maturation and condensation of the ventral nerve cord during Drosophila embryogenesis...

Research Grants63

  1. Role of Peroxidasin in Glomerular Matrix Homeostasis
    Gautam Bhave; Fiscal Year: 2013
    ..Ultimately, Dr. Bhave hopes to translate his seminal biochemical discovery into an independent line of investigation examining the role of peroxidasin in matrix remodeling and expansion in renal disease. ..
  2. Matrix Otopathology
    MICHAEL ANNE NMI GRATTON; Fiscal Year: 2011
    ..The mouse serves as a model of basement membrane abnormality and therefore data obtained will be relevant to other disease conditions which have thickened strial capillary basement membranes and hearing loss. ..
  3. Genetically testing interactions of ER and oxidative stresses in retinal disease
    Douglas Gould; Fiscal Year: 2013
    ..Knowledge of the relative roles of ER and oxidative stress can be exploited in development of targeted therapeutics that could prevent or reduce vision loss in millions of people worldwide. ..
  4. FEASIBILITY OF REPAIRING GBM DEFECTS IN VIVO
    Jeffrey H Miner; Fiscal Year: 2012
    ..The overall goal of this proposal is to use a mouse model system to investigate whether an abnormal GBM composition can be normalized in patients and whether this will improve and/or prolong kidney function. ..
  5. Extracellular Scaffold Elasticity and Binding Sites in Acinar Differentiation
    Melinda Larsen; Fiscal Year: 2013
    ..Abbreviations: AFM, atomic force microscopy;Col IV, collagen type IV;ECM, extracellular matrix;GFP, green fluorescent protein;IKVAV, Isoleucine-Lysine-Valine-Alanine-Valine;PEG, ..
  6. Nicotine and Chronic Kidney Disease
    Edgar A Jaimes; Fiscal Year: 2010
    ..We will use a variety of state of the art techniques to assess the mechanisms by which nicotine induces renal injury. ..
  7. Invasive Behavior of Tumor Cells Producing Collagenase-1
    Constance E Brinckerhoff; Fiscal Year: 2013
    ..Inhibiting MMP-1 gene expression may, therefore, be useful in therapeutically controlling melanoma metastasis. ..
  8. SIGNALING MECHANISMS IN REGULATION OF TUMOR ANGIOGENESIS
    DOMINIC E COSGROVE; Fiscal Year: 2012
    ..The data being developed from these proposed studies have potential applications to cure solid tumor growth (cancers) in which angiogenesis contributes to the disease phase. ..
  9. QD-BRET nanosensors for protease detection and imaging
    Jianghong Rao; Fiscal Year: 2012
    ..A sophisticated understanding of the differences of enzyme activity between tumor and normal tissues in living subjects will advance our understanding of cancer metastasis and help develop antimetastasis therapy. ..
  10. MOLECULAR ASPECTS OF ALPORT RENAL DISEASE PROGRESSION
    DOMINIC E COSGROVE; Fiscal Year: 2013
    ..Currently the 1 in 20,000 people with the disease are treated with ACE inhibitors, dialysis, and transplant. This work utilizes animal models to explore new therapeutic targets aimed at arresting the disease in its early stages. ..
  11. Collagen Diversity and Pathobiology in Skeletal Tissues
    David R Eyre; Fiscal Year: 2013
    ..With this knowledge new targets for therapy and prevention of genetic and acquired human disorders of bones and joints are predicted. ..
  12. BASEMENT MEMBRANE SELF-ASSEMBLY AND STRUCTURE
    PETER DANA YURCHENCO; Fiscal Year: 2013
    ..Lm[unreadable]LN also adheres to cell surfaces. We will explore interacting sequences in LN and LE domains, dissect to role of cell adhesion, and evaluate Pierson syndrome laminin [unreadable]2LN domain mutations. ..
  13. Assembly of type IV collagen in health and disease
    Raghu Kalluri; Fiscal Year: 2013
    ..Such studies will provide further insights into possible cell based therapy options for Alport syndrome, and will also provide a basic understanding of type IV collagen assembly in the GBM. ..
  14. Dysregulation of 3-prolyl-hydroxylation in Human Skeletal Dysplasias
    BRENDAN HL LEE; Fiscal Year: 2010
    ..e., the in vivo phenotypic and biochemical consequences of dysregulation of the 3-prolyl-hydroxylation machinery. ..
  15. Elucidating Key Mechanisms Regulating Cell Invasion In Vivo
    David R Sherwood; Fiscal Year: 2013
    ....
  16. MMPs contribute to hyperthermia-enhanced vascular injury
    Chen Xu Wang; Fiscal Year: 2011
    ..Hyperthermia also exacerbates the losses of collagen type IV, a major structural protein of the microvascular basal lamina, likely through the increasing activities of ..
  17. Genetically testing mechanisms of ocular development and disease
    Douglas Gould; Fiscal Year: 2013
    ..The experiments outlined in this proposal take advantage of valuable and unique resources and will provide important new insights into the mechanisms of normal ocular development and pathogenic pathways. ..
  18. PA/PLASMIN/GELATINASE CASCADE IN DIABETIC NEPHROPATHY
    WILLIAM BARICOS; Fiscal Year: 2000
    ....
  19. 92K-GL (MMP 9) AND THE INVASIVE PHENOTYPE
    MARTIN BODDEN; Fiscal Year: 1999
    ..The combination of the studies proposed in this application will provide a better understanding of the role of 92K-GL and TIMP-l, as well as other MMPs, in the invasive phenotype of human cancers of oral and other origins. ..
  20. TYPE IV COLLAGEN IN MELANOMA CELL INVASION & METASTASIS
    James McCarthy; Fiscal Year: 1999
    ..In addition, these studies may provide a first generation of structurally constrained compounds that could be useful in metastasis management in vivo. ..
  21. ANTI-GLOMERULAR BASEMENT MEMBRANE DISEASE
    Raghu Kalluri; Fiscal Year: 2001
    ..Successful completion of this project should provide new experimental information regarding the processes that govern the pathogenesis of human anti-GBM disease. ..
  22. Impact of Alport syndrome mutations and natural interruptions on collagen folding
    EILEEN HWANG; Fiscal Year: 2012
    ..The third aim will be to test small molecule chaperones to determine whether they can correct folding defects caused by missense mutations. ..
  23. Exploration and development of antiangiogenic peptides for breast cancer
    Aleksander S Popel; Fiscal Year: 2010
    ..classes of proteins: thrombospondin type I repeat-containing proteins;CXC chemokines;and alpha5 fibrils of collagen type IV. We have performed careful in vitro screening of the identified peptides using endothelial cell proliferation ..
  24. Molecular Heparan Sulfate Delivery in Guided Tissue Regeneration
    Arthur DeCarlo; Fiscal Year: 2007
    ..sulfate proteoglycan-2, is now known to be an important component of all basement membranes (along with collagen type IV and laminin)...
  25. LOCALIZATION OF PROCOLLAGENS IN DENTAL TISSUES
    CHARLES LEBLOND; Fiscal Year: 1990
    ..The aim of our work is to utilize immunolabeling for these substances using 5 nm gold particles to determine how they related to the amyloid P core of microfibrils and basotubules...
  26. Structure-Function of Type IV Collagen NC1 Domains
    Munirathinam Sundaramoorthy; Fiscal Year: 2006
    ..The accomplishment of these aims requires the application of macromolecular crystallography, computational biology, protein chemistry and molecular biology. ..
  27. STUDIES ON THE STRUCTURE OF BASEMENT MEMBRANES
    Billy Hudson; Fiscal Year: 2009
    ..abstract_text> ..
  28. LEUKOCYTE INTERACTIONS WITH EXTRACELLULAR MATRIX
    ARTHUR MERCURIO; Fiscal Year: 1999
    ....
  29. GLYCOSYLATED PROTEINS--DEVELOPING DIABETIC KIDNEY
    Edward Skolnik; Fiscal Year: 1993
    ..cachectin/tumor necrosis factor (TNF), and platelet-derived growth factor) and for matrix glycoproteins (collagen type IV and fibronectin); 2) to determine whether mesangial cells, like macrophages, contain a specific receptor for ..
  30. COLLAGEN STRUCTURAL MODULATION OF TUMOR BEHAVIORS
    GREGG FIELDS; Fiscal Year: 2002
    ..The manner in which the protein three-dimensional structure impacts protein-protein interactions, such as receptor binding to ligand, and subsequent signaling activities will also be better understood. ..
  31. FUNCTIONAL IMPORTANCE OF DOMAIN OF TYPE IV COLLAGEN
    PHOTINI TSILIBARY; Fiscal Year: 1992
    ..IV collagen. These studies will examine the possibility that supromolecular assembly of t.IV collagen in altered following non-enzymatic glucosylation...
  32. REGULATION OF COLLAGEN GENES COL4A5 AND COL4A6
    Yoav Segal; Fiscal Year: 2000
    ..It will pose a series of strategic challenges which will provide excellent background for launching an independent career. ..
  33. BASEMENT MEMBRANE THICKENING IN DIABETIC RETINOPATHY
    Sayon Roy; Fiscal Year: 2002
    ..demonstrated that in diabetes there is increased production of the basement membrane proteins fironectin and collagen type IV, the aim is to interfere with such excess production...
  34. Urinary Collagen IV in Diabetic Glomerulosclerosis
    CLYDE SHEARMAN; Fiscal Year: 2003
    ..abstract_text> ..
  35. Role of Matrix Metalloproteinase-2 in Liver Fibrosis
    Meena Bansal; Fiscal Year: 2007
    ..of hepatic stellate cells which orchestrate a shift in the normal extracellular (ECM) comprised of mostly collagen type IV to a high-density interstitial matrix composed of predominantly type I collagen...
  36. Structure, Function, and Evolution of Dr adhesins
    Stephen Moseley; Fiscal Year: 2007
    ..coli, and will provide knowledge applicable to the development of new strategies for therapy and prevention. ..
  37. SPECIFICITY OF HUMAN AUTOANTIBODIES TO PROTEOGLYCANS
    Howard Fillit; Fiscal Year: 1992
    ..Finally, the investigators propose that cell-mediated immunity causes vascular injury via T-cell recognition of PHS sites which may differ from PHS epitopes recognized by autoantibody...
  38. Molecular Analysis of Human Anti-GBM Antibodies
    Michael Madaio; Fiscal Year: 2005
    ..If successful, the strategy has the potential for application in the development of therapeutic strategies in other Ab-mediated diseases. ..
  39. KERATINOCYTE COLLAGENASE AND INHIBITOR
    Henning Birkedal Hansen; Fiscal Year: 1990
    ....
  40. THE ROLE OF ELASTIN IN THE PATHOGENESIS OF HYPERTENSION
    Charles Boyd; Fiscal Year: 1993
    ..This work will provide a solid basis for further structural studies and will provide specific probes for investigating changes in basement membranes caused by pathological conditions such as are found in diabetes...
  41. ROLE OF THE NEUROEPITHELIAL BASAL LAMINA IN NEURULATION
    Bruce Carlson; Fiscal Year: 1991
    ....
  42. METALLOPROTEINASE IN HORMONE DEPENDENT BREAST CANCER
    Y Shi; Fiscal Year: 1999
    ....
  43. A Nanofibrous Biocomposite Small-Diameter Graft
    Matthew Phaneuf; Fiscal Year: 2006
    ..Potentially, the annual market value for an "off-the-shelf" synthetic arterial bypass graft could exceed $1.5 billion. ..
  44. Mouse Model of X-Linked Alport Syndrome
    Yoav Segal; Fiscal Year: 2003
    ..abstract_text> ..
  45. ANTIBODIES TO HUMAN TYPE IV COLLAGENASE & ITS INHIBITOR
    Chi Ming Liang; Fiscal Year: 1992
    ..These molecules will be tested for potential therapeutic uses in wound healing, angiogenesis, treatment of rheumatoid arthritis and prevention of cancer metastasis...
  46. Role of Complement in Vascular Diabetic Complications
    Jose Halperin; Fiscal Year: 2005
    ..tissues releases growth factors and cytokines that stimulate cell proliferation and induce synthesis of collagen type IV by glomerular mesangial cells...
  47. FUNCTION OF EXTRACELLULAR MATRIX IN ANGIOGENESIS
    Roberto Nicosia; Fiscal Year: 1993
    ..3. To evaluate the effect of fibronectin, laminin and/or type IV collagen on the attachment, spreading and proliferation of isolated endothelial cells in gels of fibrin or type I collagen...
  48. DIABETES AND SIGNAL TRANSDUCTION ABNORMALITIES
    John Williamson; Fiscal Year: 2000
    ..This information will be critical for the development of future pharmacological approaches for the prevention and treatment of diabetic nephropathy. ..
  49. Function of CMG-2, an Anthrax Toxin Receptor
    George Davis; Fiscal Year: 2005
    ..The vWF domain of CMG-2 was shown by us to bind the extracellular matrix ligands laminin and collagen type IV and recently was shown to bind protective antigen (PA83), the known ATR binding component of the tripartite ..
  50. MOLECULAR BASIS OF X-LINKED ALPORT SYNDROME
    Jing Zhou; Fiscal Year: 1999
    ..In the longer term, an understanding of the molecular pathology of the disease may lead to opportunities for delaying the progress of the disease and, ultimately, may suggest methods for genetic therapy. ..
  51. MOLECULAR MECHANISMS OF RENAL INJURY
    Michael Madaio; Fiscal Year: 2002
    ..Hypersensitive sites identified as LCRs can then e explored at the level of the nucleosome in subsequent, second-order studies. ..
  52. BIOLOGICAL FUNCTIONS OF ENTACTIN
    ALBERT CHUNG; Fiscal Year: 2001
    ..It is hoped that the results obtained will contribute to our understanding of basal lamina function at the molecular level in normal development and in pathological conditions. ..
  53. HUMAN COLLAGENASE IV AND ITS INHIBITORS
    Robert Bird; Fiscal Year: 1991
    The degradation of the collagen type IV network in basement membranes is a critical event in many normal and pathological conditions including tissue remodelling, wound healing, tumor cell metastasis, and angiogenesis...
  54. STRUCTURAL COMPOSITION-ORGANIZATION OF BASEMENT MEMBRANE
    Heinz Furthmayr; Fiscal Year: 1991
    ..It furthermore will allow to address the question of mechanisms by which cells residing on the basal lamina can maintain their differentiated state or which lead to abnormal growth...
  55. Microenvironmental Control of the Radiation Response
    Adam Dicker; Fiscal Year: 2007
    ..The use of these tools in organotypic reconstructs promises to provide novel information about cell survival signaling in an 'in vivo' context with clear implications for therapeutic intervention. ..
  56. Role of a3a4a5(IV) collagen in podocyte behavior
    Corina Borza; Fiscal Year: 2005
    ..abstract_text> ..
  57. Integrity of the Pulmonary Blood-Gas Barrier
    John West; Fiscal Year: 2007
    ..In addition, at the more basic level, this research is devoted to a fundamental biological dilemma of the lung, namely how the blood-gas barrier combines extreme thinness with immense strength. [unreadable] [unreadable]..
  58. Point-of-Care Measurement of Glycated Hemoglobin
    MARGO COHEN; Fiscal Year: 2004
    ....
  59. LDL Modification in Diabetic Complications
    MARGO COHEN; Fiscal Year: 2007
    ..unreadable] [unreadable] [unreadable]..
  60. Reducing Renal TGF-B in Diabetic Glomerulosclerosis
    MARGO COHEN; Fiscal Year: 2005
    ..abstract_text> ..
  61. GLOMERULAR PERMSELECTIVITY IN ALPORT SYNDROME
    Clifford Kashtan; Fiscal Year: 2004
    ..This trial will compare urinary protein levels, kidney function, and renal structural changes in treated and untreated dogs. ..
  62. LAMININ IN DEVELOPING GLOMERULAR BASEMENT MEMBRANES
    Dale Abrahamson; Fiscal Year: 2003
    ..These studies will provide fundamentally new information on glomerular development and maintenance. ..
  63. Age-related structural alterations in loss of self-tolerance to a3(IV) collagen
    Dorin Bogdan Borza; Fiscal Year: 2007
    ..New targets for prevention or therapeutic intervention may be identified as a result. [unreadable] [unreadable] [unreadable] [unreadable]..