ACTN4

Summary

Gene Symbol: ACTN4
Description: actinin alpha 4
Alias: ACTININ-4, FSGS, FSGS1, alpha-actinin-4, focal segmental glomerulosclerosis 1, non-muscle alpha-actinin 4
Species: human

Top Publications

  1. ncbi Cyclosporine A and chlorambucil in the treatment of idiopathic focal segmental glomerulosclerosis
    Peter Heering
    Klinik fur Nephrologie und Rheumatologie, Heinrich Heine Universitat Dusseldorf, Dusseldorf, Germany
    Am J Kidney Dis 43:10-8. 2004
  2. ncbi Expression of alpha-actinin-4 in acquired human nephrotic syndrome: a quantitative immunoelectron microscopy study
    Nigel P Goode
    Renal Research Unit, Clinical Sciences Building, St James s University Hospital, Leeds LS9 7TF, UK
    Nephrol Dial Transplant 19:844-51. 2004
  3. pmc An interaction between alpha-actinin and the beta 1 integrin subunit in vitro
    C A Otey
    Department of Cell Biology and Anatomy, University of North Carolina, Chapel Hill 27599
    J Cell Biol 111:721-9. 1990
  4. pmc Actinin-4, a novel actin-bundling protein associated with cell motility and cancer invasion
    K Honda
    Pathology Division, National Cancer Center Research Institute, Tokyo 104, Japan
    J Cell Biol 140:1383-93. 1998
  5. ncbi Mutations in ACTN4, encoding alpha-actinin-4, cause familial focal segmental glomerulosclerosis
    J M Kaplan
    Renal and Brigham and Women s Hospital, Harvard Medical School, Boston, Massachusetts, USA
    Nat Genet 24:251-6. 2000
  6. ncbi CLP-36 PDZ-LIM protein associates with nonmuscle alpha-actinin-1 and alpha-actinin-4
    T Vallenius
    Haartman Institute and Biocentrum Helsinki, University of Helsinki, 00014 Helsinki, Finland
    J Biol Chem 275:11100-5. 2000
  7. ncbi Interactions of a hemidesmosome component and actinin family members
    A M Gonzalez
    Department of Cell and Molecular Biology, Northwestern University Medical School, 303 E Chicago Avenue, Chicago, IL 60611, USA
    J Cell Sci 114:4197-206. 2001
  8. ncbi Interaction of two actin-binding proteins, synaptopodin and alpha-actinin-4, with the tight junction protein MAGI-1
    Kevin M Patrie
    Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, 48109 0650, USA
    J Biol Chem 277:30183-90. 2002
  9. ncbi E-cadherin regulates the association between beta-catenin and actinin-4
    Yasuharu Hayashida
    Chemotherapy Division and Cancer Proteomics Project, National Cancer Center Research Institute, Tokyo, Japan
    Cancer Res 65:8836-45. 2005
  10. ncbi Alpha-actinin 4 potentiates myocyte enhancer factor-2 transcription activity by antagonizing histone deacetylase 7
    Sharmistha Chakraborty
    Department of Biochemistry, School of Medicine, Case Western Reserve University, and the Research Institute of University Hospitals of Cleveland, OH 44106, USA
    J Biol Chem 281:35070-80. 2006

Research Grants

  1. Phosphoinositide Regulation of Focal Adhesion Structure
    JEFFREY GREENWOOD; Fiscal Year: 2006
  2. Cardiotrophin-Like Cytokine 1, a Candidate Molecule for the FSGS Factor
    Virginia J Savin; Fiscal Year: 2010
  3. Friedhelm Hildebrandt; Fiscal Year: 2016
  4. MATTHEW GORDON SAMPSON; Fiscal Year: 2015
  5. Sidekick-1 Upregulation in Podocytes Induces FSGS by Disrupting MAGI-1 Function
    Lewis Kaufman; Fiscal Year: 2013
  6. Multicenter Trial of Focal Glomerulosclerosis
    Debbie Gipson; Fiscal Year: 2009
  7. Mesonephric Model of Podocyte Injury and Regeneration
    Weibin Zhou; Fiscal Year: 2012
  8. Michelle Denburg; Fiscal Year: 2016
  9. Sanja Sever; Fiscal Year: 2016
  10. Weibin Zhou; Fiscal Year: 2015

Detail Information

Publications263 found, 100 shown here

  1. ncbi Cyclosporine A and chlorambucil in the treatment of idiopathic focal segmental glomerulosclerosis
    Peter Heering
    Klinik fur Nephrologie und Rheumatologie, Heinrich Heine Universitat Dusseldorf, Dusseldorf, Germany
    Am J Kidney Dis 43:10-8. 2004
    The therapy of nephrotic syndrome in focal segmental glomerulosclerosis (FSGS) is still a matter of controversy.
  2. ncbi Expression of alpha-actinin-4 in acquired human nephrotic syndrome: a quantitative immunoelectron microscopy study
    Nigel P Goode
    Renal Research Unit, Clinical Sciences Building, St James s University Hospital, Leeds LS9 7TF, UK
    Nephrol Dial Transplant 19:844-51. 2004
    ....
  3. pmc An interaction between alpha-actinin and the beta 1 integrin subunit in vitro
    C A Otey
    Department of Cell Biology and Anatomy, University of North Carolina, Chapel Hill 27599
    J Cell Biol 111:721-9. 1990
    ..These results lead us to suggest that this integrin-alpha-actinin linkage may contribute to the attachment of actin filaments to the membrane in certain locations...
  4. pmc Actinin-4, a novel actin-bundling protein associated with cell motility and cancer invasion
    K Honda
    Pathology Division, National Cancer Center Research Institute, Tokyo 104, Japan
    J Cell Biol 140:1383-93. 1998
    ..These findings suggest that cytoplasmic actinin-4 regulates the actin cytoskeleton and increases cellular motility and that its inactivation by transfer to the nucleus abolishes the metastatic potential of human cancers...
  5. ncbi Mutations in ACTN4, encoding alpha-actinin-4, cause familial focal segmental glomerulosclerosis
    J M Kaplan
    Renal and Brigham and Women s Hospital, Harvard Medical School, Boston, Massachusetts, USA
    Nat Genet 24:251-6. 2000
    ..Here we present evidence implicating mutations in the gene encoding alpha-actinin-4 (ACTN4; ref...
  6. ncbi CLP-36 PDZ-LIM protein associates with nonmuscle alpha-actinin-1 and alpha-actinin-4
    T Vallenius
    Haartman Institute and Biocentrum Helsinki, University of Helsinki, 00014 Helsinki, Finland
    J Biol Chem 275:11100-5. 2000
    ..More generally, results presented here demonstrate that the association of PDZ-LIM proteins with the cytoskeleton extends to the actin stress fibers of nonmuscle cells...
  7. ncbi Interactions of a hemidesmosome component and actinin family members
    A M Gonzalez
    Department of Cell and Molecular Biology, Northwestern University Medical School, 303 E Chicago Avenue, Chicago, IL 60611, USA
    J Cell Sci 114:4197-206. 2001
    ..We discuss a role for BP180 in crosstalk between cell-matrix and cell-cell junctions...
  8. ncbi Interaction of two actin-binding proteins, synaptopodin and alpha-actinin-4, with the tight junction protein MAGI-1
    Kevin M Patrie
    Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, 48109 0650, USA
    J Biol Chem 277:30183-90. 2002
    ..The interaction and colocalization of MAGI-1 with two actin-bundling proteins suggest that MAGI-1 may play a role in actin cytoskeleton dynamics within polarized epithelial cells...
  9. ncbi E-cadherin regulates the association between beta-catenin and actinin-4
    Yasuharu Hayashida
    Chemotherapy Division and Cancer Proteomics Project, National Cancer Center Research Institute, Tokyo, Japan
    Cancer Res 65:8836-45. 2005
    ..Shutting down the signals mediating this association may be worth considering as a therapeutic approach to cancer invasion and metastasis...
  10. ncbi Alpha-actinin 4 potentiates myocyte enhancer factor-2 transcription activity by antagonizing histone deacetylase 7
    Sharmistha Chakraborty
    Department of Biochemistry, School of Medicine, Case Western Reserve University, and the Research Institute of University Hospitals of Cleveland, OH 44106, USA
    J Biol Chem 281:35070-80. 2006
    ..The interaction domains are mapped to C terminus of alpha-actinin 4 and amino acids 72-172 of HDAC7...
  11. ncbi Actinin-4 expression in ovarian cancer: a novel prognostic indicator independent of clinical stage and histological type
    Sohei Yamamoto
    Department of Basic Pathology, National Defense Medical College, 3 2 Namiki, Tokorozawa, Saitama, Japan
    Mod Pathol 20:1278-85. 2007
    ..Accumulation of actinin-4 in the cytoplasm may be related to a higher propensity for tumor invasiveness and metastasis, probably by enhancing cell motility, and could be a novel prognostic indicator for patients with ovarian carcinomas...
  12. doi RelA/NF-kappaB transcription factor associates with alpha-actinin-4
    Vladimir N Babakov
    Department of Cell Cultures, Institute of Cytology, St Petersburg, Russian Federation
    Exp Cell Res 314:1030-8. 2008
    ..We suggest that the alpha-actinin-4 is important for the NF-kappaB nuclear translocation and its functions inside the nucleus...
  13. pmc Motility-related actinin alpha-4 is associated with advanced and metastatic ovarian carcinoma
    Maria V Barbolina
    Department of Chemical and Biochemical Engineering, Northwestern University, Chicago, IL, USA
    Lab Invest 88:602-14. 2008
    ..The role of actinin alpha-4 (ACTN4), a cytoskeleton-associated protein implicated in the regulation of cell motility, was examined in detail...
  14. doi Expression and gene amplification of actinin-4 in invasive ductal carcinoma of the pancreas
    Satoru Kikuchi
    Chemotherapy Division, Cancer Proteomics Project, National Cancer Center Research Institute, Chuo Ku, Tokyo, Japan
    Clin Cancer Res 14:5348-56. 2008
    ..Actinin-4 is an actin-binding protein associated with enhanced cell motility, invasive growth, and lymph node metastasis. Actinin-4 might play an important role in the development and progression of pancreatic cancer...
  15. doi Actinin-4 gene amplification in ovarian cancer: a candidate oncogene associated with poor patient prognosis and tumor chemoresistance
    Sohei Yamamoto
    Department of Basic Pathology, National Defense Medical College, Tokorozawa, Japan
    Mod Pathol 22:499-507. 2009
    ..We hypothesized that the actinin-4 (ACTN4) gene might be a target of the 19q12-q13 amplicon and play an essential role of ovarian cancer progression...
  16. doi Actinin-4 expression in primary and metastasized pancreatic ductal adenocarcinoma
    Thilo Welsch
    Department of General, Visceral, and Transplant Surgery, University of Heidelberg, Heidelberg, Germany
    Pancreas 38:968-76. 2009
    ..The present study analyzed the expression of actinin-4 in the pancreas, in localized and metastasized pancreatic ductal adenocarcinoma (PDAC), and the correlation with clinical outcome...
  17. doi RNAi-mediated down-regulation of alpha-actinin-4 decreases invasion potential in oral squamous cell carcinoma
    S Yamada
    Department of Oral and Maxillofacial Surgery, Unit of Translational Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
    Int J Oral Maxillofac Surg 39:61-7. 2010
    ..The study indicated that alpha-actinin-4 could be a potential molecular target for gene therapy by RNAi targeting for OSCC...
  18. pmc Phosphorylation of alpha-actinin 4 upon epidermal growth factor exposure regulates its interaction with actin
    Hanshuang Shao
    Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15261, USA
    J Biol Chem 285:2591-600. 2010
    ..that express human epidermal growth factor receptor (EGFR) and found that both alpha-actinin 1 (ACTN1) and alpha-actinin 4 (ACTN4) were phosphorylated on tyrosine residues after stimulation with EGF, although ACTN4 was phosphorylated ..
  19. pmc Isoform-specific contributions of alpha-actinin to glioma cell mechanobiology
    Shamik Sen
    Department of Bioengineering, University of California, Berkeley, California, USA
    PLoS ONE 4:e8427. 2009
    ....
  20. doi Alpha-actinin 1 and alpha-actinin 4: contrasting roles in the survival, motility, and RhoA signaling of astrocytoma cells
    Quincy Quick
    Department of Biology, Grambling State University, Grambling, LA 71245, USA
    Exp Cell Res 316:1137-47. 2010
    ..of astrocytomas, as alpha-actinin 1 was higher in astrocytomas compared to normal brains whereas alpha-actinin 4 was elevated in high-grade astrocytomas compared to normal brains and low grade astrocytomas...
  21. doi Molecular mechanisms underlying nucleocytoplasmic shuttling of actinin-4
    Masahiro Kumeta
    Graduate School of Biostudies, Kyoto University, Kyoto 606 8501, Japan
    J Cell Sci 123:1020-30. 2010
    ....
  22. doi ACTN4 gene amplification and actinin-4 protein overexpression drive tumour development and histological progression in a high-grade subset of ovarian clear-cell adenocarcinomas
    Sohei Yamamoto
    Department of Basic Pathology, National Defense Medical College, Saitama, Japan
    Histopathology 60:1073-83. 2012
    Actinin-4, encoded by the ACTN4 gene located on chromosome 19q13.2, enhances cell motility by bundling the actin cytoskeleton...
  23. pmc Familial focal segmental glomerulosclerosis (FSGS)-linked α-actinin 4 (ACTN4) protein mutants lose ability to activate transcription by nuclear hormone receptors
    Simran Khurana
    Department of Biochemistry, School of Medicine, Case Western Reserve University and Research Institute of University Hospitals of Cleveland, Cleveland, Ohio 44106, USA
    J Biol Chem 287:12027-35. 2012
    Mutations in α-actinin 4 (ACTN4) are linked to familial forms of focal segmental glomerulosclerosis (FSGS), a kidney disease characterized by proteinuria due to podocyte injury...
  24. pmc Identification of a novel LXXLL motif in α-actinin 4-spliced isoform that is critical for its interaction with estrogen receptor α and co-activators
    Simran Khurana
    Department of Biochemistry, Case Western Reserve University and the Research Institute of University Hospitals ofCleveland, Cleveland, Ohio 44106, USA
    J Biol Chem 287:35418-29. 2012
    ..Recently, it has also become clear that ACTN4 can function in the nucleus...
  25. doi Increased expression of α-actinin-4 is associated with unfavorable pathological features and invasiveness of bladder cancer
    Hidehiko Yoshii
    Department of Urology, National Defense Medical College, Tokorozawa, Saitama 359 8513, Japan
    Oncol Rep 30:1073-80. 2013
    In the present study, the association between clinicopathological parameters and α-actinin-4 (ACTN4) expression in bladder cancer specimens was evaluated, and the functional role of ACTN4 in bladder cancer cells was investigated...
  26. doi Distinct outcome of stage I lung adenocarcinoma with ACTN4 cell motility gene amplification
    R Noro
    Division of Chemotherapy and Clinical Research, National Cancer Center Research Institute, Tokyo
    Ann Oncol 24:2594-600. 2013
    ..Even if detected at an early stage, a substantial number of lung cancers relapse after curative surgery. However, no method for distinguishing such tumors has yet been established...
  27. pmc Copy number increase of ACTN4 is a prognostic indicator in salivary gland carcinoma
    Yukio Watabe
    Division of Chemotherapy and Clinical Research, National Cancer Center Research Institute, Tokyo, 104 0045, Japan Department of Oral and Maxillofacial Surgery, Tokyo Dental College, Chiba, 261 8502, Japan
    Cancer Med 3:613-22. 2014
    Copy number increase (CNI) of ACTN4 has been associated with poor prognosis and metastatic phenotypes in various human carcinomas...
  28. ncbi Association analysis of podocyte slit diaphragm genes as candidates for diabetic nephropathy
    P Ihalmo
    Folkhalsan Institute of Genetics, Folkhalsan Research Center, University of Helsinki, Helsinki, Finland
    Diabetologia 51:86-90. 2008
    ..Here we report a genetic association analysis of four slit diaphragm genes, LRRC7, KIRREL, NPHS2 and ACTN4, in a Finnish diabetic nephropathy cohort.
  29. doi Proteomic analysis of ACTN4-interacting proteins reveals it's a putative involvement in mRNA metabolism
    Mikhail Khotin
    Department of Cell Cultures, Institute of Cytology, Russian Academy of Sciences, Tikhoretsky av, 4, 194064 St Petersburg, Russia
    Biochem Biophys Res Commun 397:192-6. 2010
    Alpha-actinin 4 (ACTN4) is an actin-binding protein. In the cytoplasm, ACTN4 participates in structural organisation of the cytoskeleton via cross-linking of actin filaments...
  30. ncbi Mutant alpha-actinin-4 promotes tumorigenicity and regulates cell motility of a human lung carcinoma
    Jeanne Menez
    Laboratoire Cytokines et Immunologie des Tumeurs Humaines, U487 INSERM, Institut Fédératif de Recherche 54, Institut Gustave Roussy, F 94805 Villejuif Cedex, France
    Oncogene 23:2630-9. 2004
    The precise role of alpha-actinin-4 encoding gene (ACTN4) is not very well understood. It has been reported to elicit tumor suppressor activity and to regulate cellular motility...
  31. pmc Disease-associated mutant alpha-actinin-4 reveals a mechanism for regulating its F-actin-binding affinity
    Astrid Weins
    Renal and Translational Medicine Divisions, Department of Medicine, Brigham and Women s Hospital and Harvard Medical School, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 104:16080-5. 2007
    ....
  32. ncbi The human non-muscle alpha-actinin protein encoded by the ACTN4 gene suppresses tumorigenicity of human neuroblastoma cells
    S N Nikolopoulos
    Department of Biological Sciences, Fordham University, Bronx, New York, NY 10458 USA
    Oncogene 19:380-6. 2000
    ..a cDNA from human neuroblastoma cell variants which encodes the second non-muscle alpha-actinin isoform designated ACTN4 (actinin-4)...
  33. ncbi Recurrent and de novo glomerular disease after renal transplantation: a report from Renal Allograft Disease Registry (RADR)
    S Hariharan
    Department of Nephrology, Medical College of Wisconsin, Milwaukee 53226, USA
    Transplantation 68:635-41. 1999
    ..A retrospective study was initiated through the Renal Allograft Disease Registry, to evaluate the prevalence and impact of recurrent and de novo diseases after transplantation...
  34. ncbi Ultrastructural changes suggestive of focal segmental glomerulosclerosis in atypical minimal change nephrotic syndrome
    M Ben Bassat
    J Casper Department of Pathology, Tel Aviv University Sackler School of Medicine, Beilinson Medical Center, Petah Tiqva, Israel
    Ultrastruct Pathol 12:1-16. 1988
    In an attempt to recognize early stages of focal segmental glomerulosclerosis (FSGS) in patients with a clinical course suggesting a diagnosis other than minimal change disease (MCD) and normal histology, or minor, nondiagnostic changes ..
  35. ncbi Cyclosporine in idiopathic nephrotic syndrome
    C Ponticelli
    Divisione Nefrologia e Dialisi, IRCCS Ospedale Maggiore, Milano, Italy
    Immunopharmacol Immunotoxicol 15:479-89. 1993
    ..On the basis of renal histology two main types of INS are recognized: minimal change nephropathy (MCN) and focal and segmental glomerular sclerosis (FSGS).
  36. ncbi Morphometric study of arterioles and glomeruli in the aging kidney suggests focal loss of autoregulation
    Gary S Hill
    Hôpital Européen Georges Pompidou and INSERM Unité 430, Paris, France
    Kidney Int 63:1027-36. 2003
    ..These observations challenge the notion of ischemia as a unitary explanation for glomerulosclerosis...
  37. ncbi Vasoactive agents modulate migration of monocytes across glomerular endothelial cells
    P C Singhal
    Department of Medicine, Long Island Jewish Medical Center, New Hyde Park, NY 11040, USA
    J Investig Med 48:110-7. 2000
    ..In both human and experimental animal models of focal glomerulosclerosis (FSGS), infiltration of macrophages in the mesangium has been considered key in the development of FSGS.
  38. ncbi The clinical effectiveness and cost-effectiveness of treatments for children with idiopathic steroid-resistant nephrotic syndrome: a systematic review
    J L Colquitt
    Southampton Health Technology Assessments Centre, University of Southampton, UK
    Health Technol Assess 11:iii-iv, ix-xi, 1-93. 2007
    ..To assess the clinical effectiveness and cost-effectiveness of treatments for children with idiopathic steroid-resistant nephrotic syndrome (SRNS)...
  39. ncbi Cell proliferation and apoptosis of the glomerular epithelial cells in rats with puromycin aminonucleoside nephrosis
    H Shiiki
    First Department of Internal Medicine, Nara Medical University, Kashihara, Nara, Japan
    Pathobiology 66:221-9. 1998
    ..epithelial cells (GECs) play an important role in the pathogenesis of focal segmental glomerulosclerosis (FSGS)...
  40. ncbi Reduced podocyte expression of alpha3beta1 integrins and podocyte depletion in patients with primary focal segmental glomerulosclerosis and chronic PAN-treated rats
    Chien An Chen
    Graduate Institute of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
    J Lab Clin Med 147:74-82. 2006
    ..and be excreted in the urine, and proteinuria is found in patients with primary focal segmental glomerulosclerosis (FSGS); both may be associated with loss of alpha3beta1integrins...
  41. ncbi Alpha-actinin-4 is required for normal podocyte adhesion
    Savita V Dandapani
    Department of Genetics, Harvard Medical School, Boston, Massachusetts, USA
    J Biol Chem 282:467-77. 2007
    Mutations in the alpha-actinin-4 gene ACTN4 cause an autosomal dominant human kidney disease...
  42. doi Modulating alpha-actinin-4 dynamics in podocytes
    Jean Louis R Michaud
    Kidney Research Centre, Ottawa Health Research Institute, University of Ottawa, Ontario, Canada
    Cell Motil Cytoskeleton 66:166-78. 2009
    ..These data suggest that dynamic regulation of alpha-actinin-4/actin interactions may be necessary for maintaining podocyte structure in response to glomerular hydrostatic forces...
  43. doi Functional analysis of promoter mutations in the ACTN4 and SYNPO genes in focal segmental glomerulosclerosis
    Shengchuan Dai
    Department of Nephrology, Ruijin Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
    Nephrol Dial Transplant 25:824-35. 2010
    To investigate the promoter mutations of ACTN4 and SYNPO genes in patients with idiopathic focal segmental glomerulosclerosis (FSGS), and to provide functional analysis of these mutations in the role of FSGS occurrence.
  44. ncbi Interaction of alpha-actinin-4 with class I PxxP motif-containing OK/SW-CL.16 protein
    Masaru Togashi
    Third Department of Internal Medicine, Akita University School of Medicine, Akita, Japan
    Nephron Exp Nephrol 107:e65-72. 2007
    ..The aim of this study was to identify novel binding partners for alpha-actinin-4 (actinin-4), an essential component of the glomerular filtration barrier...
  45. pmc Involvement of actinin-4 in the recruitment of JRAB/MICAL-L2 to cell-cell junctions and the formation of functional tight junctions
    Hiroyoshi Nakatsuji
    Department of Biochemistry, Institute of Health Biosciences, The University of Tokushima Graduate School, Tokushima 770 8503, Japan
    Mol Cell Biol 28:3324-35. 2008
    ..These results indicate that actinin-4 is involved in recruiting JRAB/MICAL-L2 to cell-cell junctions and forming functional TJs...
  46. ncbi Follow-up of steroid-resistant nephrotic syndrome: tubular proteinuria and enzymuria
    P Valles
    Instituto de Fisiopatología, Facultad de Medicina, Universidad Nacional de Cuyo, Centro Universitario 5500, Mendoza, Argentina
    Pediatr Nephrol 15:252-8. 2000
    ..in steroid-resistant nephrotic syndrome patients with a histologic diagnosis of focal segmental glomerulosclerosis (FSGS) through assessment of the urinary levels of beta 2-microglobulin (beta 2M) and N-acetyl-beta-D-glucosaminidase (..
  47. ncbi Interferon and ribavirin treatment in patients with hepatitis C-associated renal disease and renal insufficiency
    Annette Bruchfeld
    Division of Renal Medicine, Department of Clinical Science, Karolinska Institute and Huddinge University Hospital, S 141 86 Stockholm, Sweden
    Nephrol Dial Transplant 18:1573-80. 2003
    ..with or without cryoglobulinaemia, membranous glomerulonephritis (MGN) and focal segmental glomerulosclerosis (FSGS)...
  48. ncbi Actinin-4 increases cell motility and promotes lymph node metastasis of colorectal cancer
    Kazufumi Honda
    Chemotherapy Division and Cancer Proteomics Project, National Cancer Center Research Institute, 5 1 1 Tsukiji, Chuoh ku, Tokyo 104 0045, Japan
    Gastroenterology 128:51-62. 2005
    ..However, its involvement in the mechanisms of cancer invasion and metastasis remains undetermined. The current study tested the role of actinin-4 in the motility and metastatic potential of colorectal cancer cells...
  49. ncbi FSGS-associated alpha-actinin-4 (K256E) impairs cytoskeletal dynamics in podocytes
    J L R Michaud
    Kidney Research Centre and Molecular Medicine Program, Ottawa Health Research Institute, University of Ottawa, Ottawa, Ontario, Canada
    Kidney Int 70:1054-61. 2006
    Mutations in the ACTN4 gene, encoding the actin crosslinking protein alpha-actinin-4, are associated with a familial form of focal segmental glomerulosclerosis (FSGS)...
  50. doi Mice with podocyte-specific overexpression of wild type alpha-actinin-4 are healthy controls for K256E-alpha-actinin-4 mutant transgenic mice
    Jean Louis Michaud
    Kidney Research Centre, Division of Nephrology, Department of Medicine, Ottawa Health Research Institute, Ottawa Hospital and University of Ottawa, Ottawa, ON K1H 8M5, Canada
    Transgenic Res 19:285-9. 2010
    Mutations in the gene ACTN4 encoding the actin bundling protein-alpha-actinin-4 underlie an inherited form of kidney lesions known as focal segmental glomerulosclerosis (FSGS)...
  51. doi Glomerular epithelial cell injury associated with mutant alpha-actinin-4
    Andrey V Cybulsky
    Div of Nephrology, Royal Victoria Hospital, 687 Pine Ave West, Montreal, Quebec, Canada H3A1A1
    Am J Physiol Renal Physiol 297:F987-95. 2009
    Focal segmental glomerulosclerosis (FSGS) may be associated with glomerular epithelial cell (GEC; podocyte) apoptosis due to acquired injury or mutations in alpha-actinin-4...
  52. ncbi Mother-to-child transmitted WT1 splice-site mutation is responsible for distinct glomerular diseases
    E Denamur
    Laboratoire de Biochimie Génétique and Institut National de la Santé et de la Recherche Médicale U458, Hopital Robert Debre, Paris, France
    J Am Soc Nephrol 10:2219-23. 1999
    ..in patients with Frasier syndrome, another rare syndrome defined by focal and segmental glomerulosclerosis (FSGS), XY pseudohermaphroditism, and frequent occurrence of gonadoblastoma...
  53. ncbi Actinin-4 is preferentially involved in circular ruffling and macropinocytosis in mouse macrophages: analysis by fluorescence ratio imaging
    N Araki
    Department of Anatomy, Kagawa Medical University, Miki, Kagawa 761 0793, Japan
    J Cell Sci 113:3329-40. 2000
    ..A similar redistribution of actinin-4 was also observed during phagocytosis, suggesting that actinin-4 may play the same role in the two mechanistically analogous types of endocytosis, i.e. macropinocytosis and phagocytosis...
  54. ncbi Focal and segmental glomerulosclerosis in mice with podocyte-specific expression of mutant alpha-actinin-4
    Jean Louis Michaud
    Kidney Research Centre, Division of Nephrology, Department of Medicine, The Ottawa Hospital, Ottawa Health Research Institute, University of Ottawa, 451 Smyth Road, Ottawa, Ontario, Canada K1H 8M5
    J Am Soc Nephrol 14:1200-11. 2003
    Mutations in the gene encoding alpha-actinin-4 (ACTN4), an actin crosslinking protein, are associated with a form of autosomal dominant focal segmental glomerulosclerosis (FSGS)...
  55. pmc Alpha-actinin-4-mediated FSGS: an inherited kidney disease caused by an aggregated and rapidly degraded cytoskeletal protein
    June Yao
    Renal Division, Department of Medicine, Brigham and Women s Hospital and Harvard Medical School, Boston, Massachusetts, USA
    PLoS Biol 2:e167. 2004
    ..Point mutations in the alpha-actinin-4 gene ACTN4 cause an autosomal dominant form of human FSGS...
  56. ncbi Immunoexpression of podocyte-associated proteins in acquired human glomerulopathies with nephrotic syndrome
    Małgorzata Wagrowska-Danilewicz
    Department of Nephropathology, Medical University of Lodz
    Pol J Pathol 57:17-21. 2006
    ..presented with nephrotic syndrome: minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS) and nephropathy IgA (IgAN). As a control 10 biopsy specimens of the kidneys removed because of trauma were used...
  57. ncbi Outcome of renal transplantation in adolescents with focal segmental glomerulosclerosis
    Michelle A Baum
    Division of Nephrology, Children s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    Pediatr Transplant 6:488-92. 2002
    ..13-17 yr) index renal transplants to analyze differences in demographic treatment, and outcomes in adolescents with FSGS compared to other renal disease. 338 (12.6%) of adolescents had a primary diagnosis of FSGS...
  58. ncbi The relationship among nephrin, podocin, CD2AP, and alpha-actinin might not be a true 'interaction' in podocyte
    Q Fan
    Department of Pediatrics, Peking University First Hospital, Beijing, Peoples Republic of China
    Kidney Int 69:1207-15. 2006
    The abnormality of a single podocyte molecule, caused by a single gene mutation, such as NPHS1, NPHS2, CD2AP, and ACTN4, can lead to the hereditary/congenital nephrotic syndromes (NS)...
  59. doi Alpha-actinin-4 is selectively required for insulin-induced GLUT4 translocation
    Ilana Talior-Volodarsky
    Program in Cell Biology, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada
    J Biol Chem 283:25115-23. 2008
    ..amino acid labeling and mass spectrometry, we observed insulin-dependent co-precipitation of actinin-4 (ACTN4) with GLUT4 (Foster, L. J., Rudich, A., Talior, I., Patel, N., Huang, X., Furtado, L. M., Bilan, P. J., Mann, M...
  60. pmc Dynamic viscoelasticity of actin cross-linked with wild-type and disease-causing mutant alpha-actinin-4
    Sabine M Volkmer Ward
    Department of Physics and School of Engineering and Applied Sciences, Harvard University, Cambridge, Massachusetts, USA
    Biophys J 95:4915-23. 2008
    ..This is further supported by the Arrhenius-like temperature dependence of the relaxation frequencies...
  61. pmc Dense mapping of MYH9 localizes the strongest kidney disease associations to the region of introns 13 to 15
    George W Nelson
    Laboratory of Genomic Diversity, SAIC Frederick, Inc, NCI Frederick, Frederick, MD, USA
    Hum Mol Genet 19:1805-15. 2010
    ..mapping recently identified MYH9 as a susceptibility gene for idiopathic focal segmental glomerulosclerosis (FSGS), HIV-associated nephropathy (HIVAN) and end-stage kidney disease attributed to hypertension (H-ESKD) in African ..
  62. ncbi Serum glomerular permeability activity in patients with podocin mutations (NPHS2) and steroid-resistant nephrotic syndrome
    Michele Carraro
    Laboratorio di Fisiopatologia dell Uremia, Istituto G Gaslini, Largo G Gaslini 5, 16148 Genoa, Italy
    J Am Soc Nephrol 13:1946-52. 2002
    ..vitro and possibly determining proteinuria has been hypothesized in idiopathic focal segmental glomerulosclerosis (FSGS)...
  63. ncbi The Czech registry of renal biopsies. Occurrence of renal diseases in the years 1994-2000
    Ivan Rychlik
    1st Department of Medicine, 1st Faculty of Medicine, Charles University, The Czech Republic
    Nephrol Dial Transplant 19:3040-9. 2004
    ..This report describes data collected by the Czech Registry of Renal Biopsies (CRRB)...
  64. ncbi Effects of genetic polymorphisms of the renin-angiotensin system in children with nephrotic syndrome
    Yilmaz Tabel
    Department of Pediatric Nephrology, Ege University School of Medicine, Ismir, Turkey
    J Renin Angiotensin Aldosterone Syst 6:138-44. 2005
    ..Genetic polymorphisms of the various components of the RAS have been associated with differences in the clinical course of several disease states in adults and children...
  65. doi Differential chemokine expression in tubular cells in response to urinary proteins from patients with nephrotic syndrome
    Zhao Huang
    Department of Nephrology, The First Affiliated Hospital, Sun Yat Sen University, Guangzhou 510080, Guangdong, PR China
    Cytokine 42:222-33. 2008
    Comparison of urine proteins in idiopathic minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) patients has been previously conducted, but the relationship between the severity of tubular injury and the composition ..
  66. ncbi Course and prognosis of human immunodeficiency virus-associated nephropathy
    L Carbone
    Department of Medicine, Columbia University College of Physicians and Surgeons, New York, New York
    Am J Med 87:389-95. 1989
    ..rapid progression to renal insufficiency, and a morphologic pattern of focal segmental glomerulosclerosis (FSGS) on renal biopsy...
  67. ncbi Reduced endothelial factor VIII staining in renal microcirculation correlates with hemodynamic alteration in nephrosis
    Narisa Futrakul
    Physiology Department, King Chulalongkorn Memorial Hospital, Bangkok, Thailand
    Ren Fail 25:759-64. 2003
    ..with mesangial proliferation (MesP) and six nephrotic patients associated with focal segmental glomerulosclerosis (FSGS)...
  68. ncbi The effect of proteinase inhibitors on glomerular albumin permeability induced in vitro by serum from patients with idiopathic focal segmental glomerulosclerosis
    Michele Carraro
    Department of Medicina Clinica, University of Trieste, Italy
    Nephrol Dial Transplant 19:1969-75. 2004
    ..permeability alterations induced in vitro by serum from patients affected by focal segmental glomerulosclerosis (FSGS) remains unidentified...
  69. ncbi Effects of population succession on demographic and genetic processes: predictions and tests in the daylily Hemerocallis thunbergii (Liliaceae)
    Mi Yoon Chung
    Department of Biology and Institute of Basic Science, Gyeongsang National University, Jinju 660 701, South Korea
    Mol Ecol 16:2816-29. 2007
    ..To determine how demographic structure and fine-scale genetic structure (FSGS) change with stages in a population's successional history, we studied Hemerocallis thunbergii (Liliaceae), a ..
  70. ncbi Recurrence of nephrotic proteinuria in children with focal segmental glomerulosclerosis after renal transplantation treated with plasmapheresis and immunoadsorption: case reports
    F Fencl
    Department of Pediatrics, University Hospital Motol, Charles University in Prague, 2nd Medical School, Prague, Czech Republic
    Transplant Proc 39:3488-90. 2007
    Idiopathic focal segmental glomerulosclerosis (FSGS) is believed to be caused by a circulating permeability factor. FSGS recurrence is common after transplantation...
  71. ncbi Tracer studies in the rat demonstrate misdirected filtration and peritubular filtrate spreading in nephrons with segmental glomerulosclerosis
    W Kriz
    Institut fur Anatomie und Zellbiologie, Universitat Heidelberg, Heidelberg, Germany
    J Am Soc Nephrol 12:496-506. 2001
    In two genetic models of "classic" focal segmental glomerulosclerosis (FSGS), the Milan normotensive and the Fawn-hooded hypertensive rats, tracer studies were performed to test the hypothesis that misdirected glomerular filtration and ..
  72. ncbi Combination of immunosuppressive agents in treatment of steroid-resistant minimal change disease and primary focal segmental glomerulosclerosis
    Kamel El-Reshaid
    Department of Medicine, Faculty of Medicine, Kuwait University, PO Box 24923, Safat 13110, Kuwait
    Ren Fail 27:523-30. 2005
    Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are the most prevalent histopathological lesions in idiopathic nephrotic syndrome (INS)...
  73. ncbi WT1 splice-site mutations are rarely associated with primary steroid-resistant focal and segmental glomerulosclerosis
    E Denamur
    INSERM U458, Hopital Robert Debre, and Laboratoire d Anatomopathologic and EA 3102, Paris, France
    Kidney Int 57:1868-72. 2000
    ..been reported in Frasier syndrome, which is defined by the association of focal and segmental glomerulosclerosis (FSGS), male pseudohermaphroditism, and gonadoblastoma...
  74. ncbi Glomerular tip lesion in minimal change nephropathy: a study of autopsies before 1950
    Mark Haas
    Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA
    Am J Kidney Dis 39:1168-75. 2002
    ..reported that such lesions have a clinical course similar to that of primary focal segmental glomerulosclerosis (FSGS), and the tip lesion often is included among histological variants of FSGS...
  75. ncbi Race and glomerulonephritis in patients with and without hepatosplenic Schistosomiasis mansoni
    A A Lopes
    Nephrology and Epidemiology, Federal University of Bahia, Brazil
    Clin Nephrol 58:333-6. 2002
    United States investigators have shown evidence of higher susceptibility to focal segmental glomerulosclerosis (FSGS) in blacks than in whites. This association between race and FSGS has not been assessed outside the US...
  76. ncbi A case of homozygous familial hypercholesterolemia with focal segmental glomerulosclerosis
    Ahmet Midhat Elmaci
    Department of Pediatric Nephrology, School of Meram Medicine, University of Selcuk, 42080 Konya, Turkey
    Pediatr Nephrol 22:1803-5. 2007
    ..Homozygous FH occurs in only one in a million people. Focal segmental glomerulosclerosis (FSGS) is clinically characterized by proteinuria, which is marked in the majority of cases and accompanied by nephrotic ..
  77. ncbi E pluribus unum: The riddle of focal segmental glomerulosclerosis
    Alain Meyrier
    Hopital Europeen Georges Pompidou, and INSERM U 430, Paris, France
    Semin Nephrol 23:135-40. 2003
    A recent consensus conference proposed a new classification for focal segmental glomerulosclerosis (FSGS)...
  78. ncbi The focal segmental glomerulosclerosis permeability factor: biochemical characteristics and biological effects
    Mukut Sharma
    Nephrology Division, Department of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin 53226, USA
    Exp Biol Med (Maywood) 229:85-98. 2004
    Focal segmental glomerulosclerosis (FSGS) is characterized by steroid resistant nephrotic syndrome and progression to end-stage renal disease...
  79. ncbi Paraoxonase 1 192 and 55 polymorphisms in nephrotic children
    Nese Karaaslan Biyikli
    Department of Pediatric Nephrology, Marmara University School of Medicine, Tophanelioglu cd, No 13 15, 34660, Altunizade, Istanbul, Turkey
    Pediatr Nephrol 21:649-54. 2006
    ..we aimed to investigate PON1 192 and PON1 55 polymorphisms in children with focal segmental glomerulosclerosis (FSGS) and control subjects. The study included 25 children with biopsy-proven FSGS and 30 healthy controls...
  80. ncbi Immunosuppressive treatment of idiopathic focal segmental glomerulosclerosis: a five-year follow-up study
    Dimitrios S Goumenos
    Department of Internal Medicine, Division of Nephrology, University Hospital of Patras, Patras, Greece
    Nephron Clin Pract 104:c75-82. 2006
    Focal segmental glomerulosclerosis (FSGS) is a common type of glomerular disease that can lead to chronic renal failure. Various therapeutic regimens have been used in nephrotic FSGS patients...
  81. ncbi Steroid-resistant idiopathic childhood nephrosis: overdiagnosed and undertreated
    Jochen H H Ehrich
    Department of Paediatric Nephrology, Children s Hospital, Hannover Medical School, Germany
    Nephrol Dial Transplant 22:2183-93. 2007
    ..induction therapy for steroid-resistant nephrotic syndrome (SRNS) due to either focal segmental glomerulosclerosis (FSGS) or minimal change nephrotic syndrome (MCNS) has been reported to be <50%...
  82. doi Tacrolimus therapy in adults with steroid- and cyclophosphamide-resistant nephrotic syndrome and normal or mildly reduced GFR
    Xiayu Li
    Kidney Disease Center, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, People s Republic of China
    Am J Kidney Dis 54:51-8. 2009
    ..In a proportion of adults with steroid-resistant nephrotic syndrome (SRNS), intravenous cyclophosphamide therapy fails. Tacrolimus may be a promising alternative to cyclophosphamide for such patients...
  83. ncbi Genetic polymorphisms of the renin-angiotensin system and the outcome of focal segmental glomerulosclerosis in children
    Y Frishberg
    Department of Surgery A, Shaare Zedek Medical Center Jerusalem, Israel
    Kidney Int 54:1843-9. 1998
    The clinical course of primary focal segmental glomerulosclerosis (FSGS) in children is variable, with some patients having a much more rapidly progressing course than others...
  84. ncbi Cell-cycle regulatory proteins in podocyte cell in idiopathic nephrotic syndrome of childhood
    Tarak Srivastava
    Section of Nephrology and Department of Pathology, The Children s Mercy Hospital, University of Missouri at Kansas City, 64108, USA
    Kidney Int 63:1374-81. 2003
    ..In adults with cellular and collapsing focal segmental glomerulosclerosis (FSGS), the expression of cell-cycle regulatory proteins such as p27, p57, and cyclin D is decreased and expression of ..
  85. ncbi Differential binding of cross-reactive anti-DNA antibodies to mesangial cells: the role of alpha-actinin
    Zeguo Zhao
    The Irving and Ruth Claremon Research Laboratory, Division of Rheumatology, Albert Einstein College of Medicine, Bronx, NY 10461, USA
    J Immunol 176:7704-14. 2006
    ..we established that two isoforms of alpha-actinin that are present in the kidney, alpha-actinin 1 and alpha-actinin 4, can both be targeted by anti-alpha-actinin Abs...
  86. pmc Genetics of focal segmental glomerulosclerosis
    Robert P Woroniecki
    Albert Einstein College of Medicine, Bronx, NY, USA
    Pediatr Nephrol 22:638-44. 2007
    The recent advances in understanding the pathophysiology of focal segmental glomerulosclerosis (FSGS) and molecular function of glomerular filtration barrier come directly from genetic linkage and positional cloning studies...
  87. ncbi A prospective, open-label trial of sirolimus in the treatment of focal segmental glomerulosclerosis
    James A Tumlin
    Department of Medicine, Division of Nephrology, Emory University School Medicine, 1639 Pierce Drive, Atlanta, GA 30322, USA
    Clin J Am Soc Nephrol 1:109-16. 2006
    Calcineurin inhibitors are effective therapy for steroid-resistant focal segmental glomerulosclerosis (FSGS) but are associated with significant morbidity and nephrotoxicity...
  88. ncbi Alpha-actinin-1 phosphorylation modulates pressure-induced colon cancer cell adhesion through regulation of focal adhesion kinase-Src interaction
    David H Craig
    Department of Surgery, John D Dingell Veterans Affairs Medical Center, Wayne State University, and Karmanos Cancer Institute, Detroit, MI, USA
    Am J Physiol Cell Physiol 293:C1862-74. 2007
    ....
  89. ncbi Fractional excretion of high- and low-molecular weight proteins and outcome in primary focal segmental glomerulosclerosis
    J K Deegens
    Department of Nephrology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
    Clin Nephrol 68:201-8. 2007
    Predicting prognosis in patients with a nephrotic syndrome due to primary FSGS remains difficult. Recently, it was suggested that the fractional excretion (FE) of IgG (threshold 0...
  90. doi Elevated asymmetric dimethylarginine (ADMA) and inverse correlation between circulating ADMA and glomerular filtration rate in children with sporadic focal segmental glomerulosclerosis (FSGS)
    Thomas Lücke
    Department of Paediatric, Kidney, Liver and Metabolic and Nephrologic Diseases, Hannover Medical School, Carl Neuberg Str 1, D 30625, Hannover, Germany
    Nephrol Dial Transplant 23:734-40. 2008
    Steroid-resistant nephrotic syndromes (NS) with focal and segmental glomerulosclerosis (FSGS) can be differentiated into sporadic and syndromic forms...
  91. pmc Rapid recycling of beta-adrenergic receptors is dependent on the actin cytoskeleton and myosin Vb
    Ellen E Millman
    Department of Pediatrics, Baylor College of Medicine, 6621 Fannin, CCC 1040 00, Houston, TX 77030, USA
    Traffic 9:1958-71. 2008
    ..These results indicate that the actin cytoskeleton is required for the efficient recycling of beta(2)ARs, a process that likely is dependent on myosin Vb...
  92. pmc An essential role of the universal polarity protein, aPKClambda, on the maintenance of podocyte slit diaphragms
    Tomonori Hirose
    Department of Molecular Biology, Yokohama City University Graduate School of Medical Science, Yokohama, Kanagawa, Japan
    PLoS ONE 4:e4194. 2009
    ..disassembly of slit diaphragms, a disturbance in apico-basal cell polarity, and focal segmental glomerulosclerosis (FSGS)...
  93. doi The MLL recombinome of adult CD10-negative B-cell precursor acute lymphoblastic leukemia: results from the GMALL study group
    Thomas Burmeister
    Charite Universitatsmedizin Berlin, Campus Benjamin Franklin CBF, Medizinische Klinik III, Berlin, Germany
    Blood 113:4011-5. 2009
    ..MLL fusion genes were found: 2 MLL-TET1 cases, not previously reported in ALL, 1 MLL-AF9, 1 MLL-PTD, a novel MLL-ACTN4, and an MLL-11q23 fusion...
  94. ncbi Resolution of nephrotic syndrome after successful bariatric surgery in patient with biopsy-proven FSGS
    Y Huan
    Renal and Pathology Divisions, University of Pennsylvania, Philadelphia, PA, USA
    Clin Nephrol 71:69-73. 2009
    ..with morbid obesity, proteinuria and renal biopsy findings of focal global and segmental glomerulosclerosis (FSGS), which can be associated with significant renal impairment...
  95. pmc Follow-up of phase I trial of adalimumab and rosiglitazone in FSGS: III. Report of the FONT study group
    Alexandra Peyser
    Department of Pediatrics, Division of Nephrology, Schneider Children s Hospital of North Shore LIJ Health System, 269 01 76th Avenue, New Hyde Park, NY 11040, USA
    BMC Nephrol 11:2. 2010
    Patients with resistant primary focal segmental glomerulosclerosis (FSGS) are at high risk of progression to chronic kidney disease stage V. Antifibrotic agents may slow or halt this process...
  96. ncbi A female infant with Frasier syndrome showing splice site mutation in Wilms' tumor gene (WT1) intron 9
    S Fujita
    Department of Pediatrics, Kinki University School of Medicine, Osaka Sayama, Japan
    Clin Nephrol 73:487-91. 2010
    ..On renal biopsy, she was diagnosed with focal segmental glomerulosclerosis (FSGS)...
  97. ncbi Frequency of primary glomerular disease in northeastern China
    Yi Qi Wu
    Department of Pathology, Harbin Medical University, Heilongjiang, China
    Braz J Med Biol Res 44:810-3. 2011
    ..membranous nephropathy (MN), membranoproliferative glomerulonephritis (MPGN), focal segmental glomerulosclerosis (FSGS), and endocapillary proliferative glomerulonephritis (EnPGN). MCD was the disease most frequently observed (43...
  98. doi Podocyte Wnt/ß-catenin pathway is activated by integrin-linked kinase in clinical and experimental focal segmental glomerulosclerosis
    Marcelo A Naves
    Nephrology Division, Department of Medicine, Federal University of Sao Paulo UNIFESP, Sao Paulo, Brazil
    J Nephrol 25:401-9. 2012
    ..Here, we investigated ILK expression as well as its involvement with the Wnt signaling pathway in renal biopsies of patients with primary focal segmental glomerulosclerosis (FSGS), and in a correspondent in vivo model of podocyte lesion.
  99. pmc Urinary MMP-9/NGAL ratio as a potential marker of FSGS in nephrotic children
    Agata Korzeniecka-Kozerska
    Department of Paediatrics and Nephrology, Medical University of Białystok, Białystok, Poland
    Dis Markers 34:357-62. 2013
    ..new markers for distinguishing minimal change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS) in children...
  100. ncbi Paraneoplastic focal segmental glomerulosclerosis in a patient with lung adenocarcinoma
    Akira Tadokoro
    Department of Internal Medicine, Kagawa University, Japan
    Intern Med 52:1953-6. 2013
    Focal segmental glomerulosclerosis (FSGS) is extremely rare among paraneoplastic nephrotic syndromes. We herein report a case of lung adenocarcinoma with nephrotic syndrome caused by paraneoplastic FSGS...
  101. pmc A multicolor podocyte reporter highlights heterogeneous podocyte changes in focal segmental glomerulosclerosis
    Jianling Tao
    1 Renal Electrolyte and Hypertension Division, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA 2 Division of Nephrology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China
    Kidney Int 85:972-80. 2014
    In contrast to most glomerular diseases, the injury pattern in focal segmental glomerulosclerosis (FSGS) is highly heterogeneous, even though podocytes are genetically identical and exposed to the same environmental factors...

Research Grants71

  1. Phosphoinositide Regulation of Focal Adhesion Structure
    JEFFREY GREENWOOD; Fiscal Year: 2006
    ..abstract_text> ..
  2. Cardiotrophin-Like Cytokine 1, a Candidate Molecule for the FSGS Factor
    Virginia J Savin; Fiscal Year: 2010
    DESCRIPTION (provided by applicant): Steroid resistant nephrotic syndrome and focal segmental glomerulosclerosis (FSGS) pose especially difficult clinical problems because the disease recurs in about 30% of such patients after renal ..
  3. Friedhelm Hildebrandt; Fiscal Year: 2016
    DESCRIPTION (provided by applicant): Discover and functionally characterize full-penetrance causes of nephrosis/FSGS. Chronic kidney diseases (CKD) take one of the highest tolls on human health, and their prevalence has been rising in ..
  4. MATTHEW GORDON SAMPSON; Fiscal Year: 2015
    ..of primary NS affecting children are minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS)...
  5. Sidekick-1 Upregulation in Podocytes Induces FSGS by Disrupting MAGI-1 Function
    Lewis Kaufman; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Focal and segmental glomerulosclerosis (FSGS) is a leading cause of nephrotic syndrome and end-stage renal disease worldwide...
  6. Multicenter Trial of Focal Glomerulosclerosis
    Debbie Gipson; Fiscal Year: 2009
    DESCRIPTION (provided by applicant): Idiopathic focal segmental glomerulosclerosis (FSGS) is a progressive scarring disorder that causes proteinuria and kidney failure in the majority of affected individuals...
  7. Mesonephric Model of Podocyte Injury and Regeneration
    Weibin Zhou; Fiscal Year: 2012
    ..and no effective therapy is available for the disease, which results in focal segmental glomerular sclerosis (FSGS) and leads to end-stage kidney disease (ESKD)...
  8. Michelle Denburg; Fiscal Year: 2016
    ..preliminary work demonstrated that glomerular disease, particularly focal segmental glomerulosclerosis (FSGS), was an independent risk factor for low total and free 25(OH) D levels and vitamin D-binding protein (DBP) in ..
  9. Sanja Sever; Fiscal Year: 2016
    ..1) mouse model in which actin cytoskeleton is mis- regulated due to expression of 'gain of function'1-actinin 4 mutant;(2) mice lacking CD2AP (adaptor protein involved in regulation of actin cytoskeleton and signaling in ..
  10. Weibin Zhou; Fiscal Year: 2015
    ..and no effective therapy is available for the disease, which results in focal segmental glomerular sclerosis (FSGS) and leads to end-stage kidney disease (ESKD)...
  11. Glucocorticoid Receptor Function in Steroid Resistant Childhood Nephrotic Syndrom
    JORGE A INIGUEZ LLUHI; Fiscal Year: 2010
    ..a collaborative arrangement with both the Midwest Pediatric Nephrology Consortium and the ongoing NIH-sponsored FSGS clinical trial, we propose to analyze the mechanistic link between the clinical response to glucocorticoids and ..
  12. Jochen Reiser; Fiscal Year: 2014
    ..of the pathobiology of TRPC6-related human kidney diseases including Focal Segmental Glomerulosclerosis (FSGS) and promote the development of anti-proteinuric drugs interfering with TRPC6 and its cellular effects on podocytes...
  13. Genetics of Renal End Organ Damage in Hypertension
    Michael R Garrett; Fiscal Year: 2013
    ..in the Dahl salt- sensitive (S) rat, which exhibits significant proteinuria and focal segmental glomerulosclerosis (FSGS). An initial genetic analysis for proteinuria and renal injury was performed using a population derived from the S...
  14. TRPC-mediated calcium signaling in podocytes
    Anna Greka; Fiscal Year: 2012
    ..of proteinuria, and thus its potential as a new therapeutic target for diseases such as nephrotic syndrome, FSGS, diabetic and hypertensive nephropathy...
  15. FSGS PERMEABILITY FACTOR IN CHILDHOOD NEPHROTIC SYNDROME
    Laurence Greenbaum; Fiscal Year: 1999
    ..The specter of FSGS is present when nephrotic syndrome develops in children, though most have a good prognosis...
  16. KIMBERLY JEAN REIDY; Fiscal Year: 2015
    ..novel pathways that may provide insight into mechanisms of proteinuric kidney disease and focal glomerulosclerosis (FSGS), a leading cause of childhood CKD and ESKD...
  17. Alessia Fornoni; Fiscal Year: 2015
    DESCRIPTION (provided by applicant): Recurrent focal segmental glomerulosclerosis (FSGS) after transplantation is a highly prevalent condition where rituximab (a monoclonal antibody against CD20) may have a potential indication...
  18. A New Locus for Hereditary FSGS on Chromosome 2p
    RASHEED ADEBAYO GBADEGESIN; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Research: Focal and segmental glomerulosclerosis (FSGS) is an important clinicopathologic entity, with an incidence that is estimated at seven per million individuals...
  19. William E Smoyer; Fiscal Year: 2016
    ..with four glomerular diseases (Minimal Change Nephrotic Syndrome [MCD], Focal Segmental Glomerulosclerosis [FSGS], IgA Nephropathy [IgAN], and Idiopathic Membranous Nephropathy[IMN]) into a longitudinal cohort study that will ..
  20. Martin R Pollak; Fiscal Year: 2016
    ..by applicant): Identification of the first several nephrotic syndrome and focal segmental glomerulosclerosis (FSGS) genes has had a significant impact on the understanding of glomerular function and disease...
  21. Tacrolimus and Plasmapheresis in Treatment-Resistant FSGS
    Tej Mattoo; Fiscal Year: 2007
    unreadable] DESCRIPTION (provided by applicant): Focal segmental glomerulosclerosis (FSGS) is observed in 10-20% of children and 5% of adults with idiopathic nephrotic syndrome, and in 15% of children and 5% of adults with end-stage ..
  22. Comparison of Cyclosporine A and Mycophenolate in FSGS
    Ronald Hogg; Fiscal Year: 2005
    ..of treatment options for children and young adults with nephrotic syndrome and focal segmental glomerulosclerosis (FSGS)...
  23. Matthias Kretzler; Fiscal Year: 2016
    Minimal Change Disease (MCD), Focal and Segmental Glomerulosclerosis (FSGS), and Membranous Nephropathy (MN) are glomerular diseases that despite their rarity, account for a large fraction of prevalent ESRD...
  24. Alpha actinin 4: its functions and regulation
    Hung Ying Kao; Fiscal Year: 2013
    ..Genetic mutations in ACTN4 disrupt cytoskeletal structure of the podocytes and have been linked to several glomerular diseases...
  25. Virginia J Savin; Fiscal Year: 2014
    DESCRIPTION (provided by applicant): Summary: Focal segmental glomerulosclerosis (FSGS) is the most common primary glomerular cause of chronic renal failure requiring dialysis and transplantation...
  26. 6th International Podocyte Conference
    Stuart Shankland; Fiscal Year: 2006
    ..The leading causes of nephrotic syndrome in adults includes focal segmental glomerulosclerosis (FSGS), membranous nephropathy, minimal change disease and diabetic nephropathy...
  27. PODOCYTURIA IN FOCAL SEGMENTAL GLOMERULOSCLEROSIS
    DAVID KURNIT; Fiscal Year: 2005
    ..of the glomerular filter on a molecular level with the identification of nephrin, podocin, and alpha-actinin 4 ...
  28. Role of KLF15 in podocyte differentiation
    Sandeep Mallipattu; Fiscal Year: 2012
    ..podocyte differentiation has been implicated in many kidney diseases such as Focal Segmental Glomerular Sclerosis (FSGS) and HIV Associated Nephropathy (HIVAN)...
  29. The Role of Polyomaviruses in Pediatric Renal Diseases
    JOHN VANCHIERE; Fiscal Year: 2006
    ..Based on human and animal studies, others have linked SV40 to focal segmental glomerulosclerosis (FSGS), an enigmatic kidney disease that is increasing in incidence in both children and adults...
  30. Identification of The Membranous Nephropathy Antigen
    David Salant; Fiscal Year: 2005
    ..of MN as compared with other common causes of nephrotic syndrome, namely focal and segmental glomerulosclerosis (FSGS) and diabetic nephropathy (DN)...
  31. Role of the novel gene fam4b in phosphatidylserine synthesis in podocytes and mai
    HUA A LU; Fiscal Year: 2012
    ..from mice with proteinuric glomerular dysfunction and from patients with focal segmental glomerulosclerosis (FSGS). From a yeast two-hybrid screen we identified phosphatidylserine synthase-1 (PSS1) as a Fam49b interacting partner...
  32. 5TH INTERNATIONAL PODOCYTE CONFERENCE
    Stuart Shankland; Fiscal Year: 2004
    ..NS is characterized by disease/injury to the podocyte as occurs in FSGS, membranous nephropathy, minimal change disease and diabetic nephropathy...
  33. Martin R Pollak; Fiscal Year: 2016
    ..filtration rate, and a histologic pattern of injury characterized by focal and segmental glomerulosclerosis (FSGS). This gene, INF2 (for Inverted Formin 2), is a member of the diaphanous subgroup of the formin family...
  34. Regulation of podocyte survival by dendrin
    Kirk N Campbell; Fiscal Year: 2013
    ..Injury to these cells is an important mechanism in the progression of proteinuric kidney diseases. ..
  35. Regulation of Podocyte Actin Cytoskeleton
    Puneet Garg; Fiscal Year: 2012
    ..3. To further characterize the physiological role of cofilin in podocytes in development and in podocyte injury, we will engineer and phenotye mice deleted of cofilin in a podocyte-specific fashion. ..
  36. Characterization of TRPC6 As A Cause for Focal and Segmental Glomerulosclerosis
    Michelle P Winn; Fiscal Year: 2012
    ..Channel 6 (TRPC6) gene causing an autosomal dominant form of familial focal and segmental glomerulosclerosis (FSGS)...
  37. PETER H MUNDEL; Fiscal Year: 2015
    ..We further hypothesize that the persistence of podocyte hic-5 expression in focal segmental glomerulosclerosis (FSGS) confers a senescence phenotype, thereby promoting the progression to ESRD...
  38. FSGS Cohort Study
    Howard Trachtman; Fiscal Year: 2010
    DESCRIPTION (provided by applicant): The FSGS Cohort Study is a prospective, longitudinal cohort study designed for long-term assessment of the NIH sponsored Focal Segmental Glomerulosclerosis Clinical Trial (FSGS-CT) participants...
  39. TRPC6 Regulation and its Role in Glomerular Pathology
    JOHANNES S SCHLONDORFF; Fiscal Year: 2012
    ..PUBLIC HEALTH RELEVANCE: Focal segmental glomerular sclerosis (FSGS) is a kidney lesion seen in a number of disease states, contributes significantly to the population of patients ..
  40. ETIOLOGIC FACTOR IN FOCAL GLOMERULOSCLEROSIS
    Virginia Savin; Fiscal Year: 2000
    ..Adapted from Investigator's Abstract): Evidence that a circulating factor is responsible for proteinuria in FSGS includes early recurrence of proteinuria in renal allografts, favorable clinical responses to plasmapheresis and ..
  41. Vivian Tang; Fiscal Year: 2016
    ..Recently, we found that FSGS1/[unreadable]-actinin-4 is required for actin assembly at cell-cell contacts...
  42. Novel Therapies for Resistant FSGS
    Howard Trachtman; Fiscal Year: 2011
    Primary FSGS is a serious renal disease, accounting for nearly 10-15% of all pediatric and adult patients requiring chronic dialysis or transplantation...
  43. Jochen Reiser; Fiscal Year: 2016
    DESCRIPTION (provided by applicant): Role of Circulating suPAR in FSGS Focal Segmental Glomerulosclerosis (FSGS) is a severe glomerular disease that is characterized by podocyte injury, proteinuria and progressive renal decline, the ..
  44. ROY SOBERMAN; Fiscal Year: 2016
    ..clinical study of B7-1 blockade in patients with proteinuria, including cases of recurrent and non-recurrent FSGS. This should in the long-term enable us to develop novel therapies that tackle proteinuria and glomerulosclerosis ..
  45. Tacrolimus vs Intensive Prednisone in Pediatric FSGS
    Frederick Kaskel; Fiscal Year: 2007
    Focal Segmental Glomerulosclerosis (FSGS) is a major cause of chronic kidney disease in childhood. This glomerulopaty is more frequent in minority populations, and often recurs in the transplanted kidney...
  46. Biological Mechanism of FSGS-1
    Martin R Pollak; Fiscal Year: 2011
    DESCRIPTION (provided by applicant): Mutations in 1-actinin-4 (Actn4), an actin crosslinking protein encoded by the human ACTN4 gene, cause an autosomal dominant form of focal segmental glomerulosclerosis (FSGS)...
  47. Functional and Phenotypic Characterization of a New FSGS Gene
    RASHEED ADEBAYO GBADEGESIN; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Focal segmental glomerulosclerosis (FSGS), a common cause of kidney failure, is the result of pathogenic changes that alter the functional integrity of the glomerular filtration barrier (GFB)...
  48. Martin R Pollak; Fiscal Year: 2016
    ..different types of kidney disease including hypertension-associated ESRD, focal segmental glomerulosclerosis (FSGS), and HIV-associated nephropathy (HIVAN)...
  49. Martin R Pollak; Fiscal Year: 2016
    DESCRIPTION (provided by applicant): Mutations in 1-actinin-4 (Actn4), an actin crosslinking protein encoded by the human ACTN4 gene, cause an autosomal dominant form of focal segmental glomerulosclerosis (FSGS)...
  50. Effects of FSGS factor on arachidonic acid metabolism
    Ellen McCarthy; Fiscal Year: 2003
    DESCRIPTION (provided by candidate): Focal segmental glomerulosclerosis (FSGS) is characterized by heavy proteinuria and progression to end-stage renal disease...
  51. EICOSANOIDS IN FOCAL SEGMENTAL GLOMERULOSCLEROSIS
    Ellen McCarthy; Fiscal Year: 2002
    DESCRIPTION (adapted from the application) Focal segmental glomerulosclerosis (FSGS) is a renal disease of unknown etiology characterized by heavy proteinuria, hypertension, and progression to renal failure...
  52. Biophysical Properties of Renal Glomeruli and Podocytes
    Martin R Pollak; Fiscal Year: 2013
    ..Alport model, abnormal GBM), conditional podocyte integrin 21-/- (abnormal cytoskeletal-GBM connection), and Actn4-/- (abnormal actin cross-linking), that based on their distinct molecular pathology, will define specific ..
  53. Role of APOL1 in HIVAN pathogenesis
    Waldemar Popik; Fiscal Year: 2013
    ..Further, striking racial disparities in the frequency of focal segmentl glomerulosclerosis (FSGS) and HIVAN have led to the discovery of genetic variants of the APOL1 gene that predispose African Americans ..
  54. Katalin Susztak; Fiscal Year: 2016
    ..Diabetic and hypertensive renal disease (DNP, HN) and FSGS -so called non- immune mediated degenerative glomerular diseases- are responsible for >75% of ESRD cases in the ..
  55. p66ShcA in HIV associated Nephropathy
    Pravin C Singhal; Fiscal Year: 2013
    ..HIVAN) is a distinct clinico-pathological entity that is characterized by focal segmental glomerular sclerosis (FSGS) and tubulointerstitial lesions...
  56. Ming Jer Tsai; Fiscal Year: 2014
    ..Furthermore, adult mice with the loss of one COUP-TFII allele display polycystic kidneys, glomerulosclerosis (FSGS) and loss of kidney function, phenotypes resembling human kidney diseases...
  57. William Brieher; Fiscal Year: 2016
    ..in two of them result in the same inherited kidney disease as mutant forms of [unreadable]-actinin known as FSGS suggesting a common cell biological function...
  58. RNA profiling of HIV-associated nephropathy in patients with the MYH9 risk allele
    PAUL EVAN KLOTMAN; Fiscal Year: 2010
    ..of the gene MYH9, which encodes non-muscle myosin heavy chain IIA (Myosin-9), with idiopathic and HIV-associated FSGS in African-Americans...
  59. Glomerulosclerosis in Human FSGS and Animal Models
    S Steven Potter; Fiscal Year: 2010
    Focal Segmental Glomerulosclerosis (FSGS) is a heterogeneous and devastating disease that is one of the major causes of renal failure...
  60. Genetics and Pharmacogenetics in FSGS (PPG Project 4)
    Lisa Guay Woodford; Fiscal Year: 2007
    Idiopathic focal segmental glomerulosclerosis (FSGS) is a primary glomerular disorder that is often[unreadable] associated with refractory edema, severe infections, thromboembolic complications, and progression to[unreadable] end-stage ..
  61. BRUCE MARSHALL ROBINSON; Fiscal Year: 2016
    ..disease (GDPrime) consortium with a focus on minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), immunoglobulin A nephropathy (IGAN), and idiopathic membranous nephropathy (IMN)...
  62. ROGER CHARLES WIGGINS; Fiscal Year: 2015
    DESCRIPTION (provided by applicant): There is now strong evidence that FSGS is caused by podocyte depletion due to accelerated podocyte loss from glomeruli...
  63. SPONTANEOUS TUBULOINTERSTITAL NEPHRITIS IN KDKD MICE
    David L Gasser; Fiscal Year: 2012
    ..This disease is similar in certain respects to focal segmental glomerulosclerosis (FSGS), and there is evidence that some patients with FSGS have mutant alleles of the same gene that is defective in kd/..
  64. GENETIC EPIDEMIOLOGY OF IDIOPATHIC NEPHROTIC SYNDROME
    Abhay Vats; Fiscal Year: 2005
    ..In this study we aim to evaluate mutations in several candidate genes (including nephrin, and actinin 4) located on chromosome 19q13 in autosomal dominant NS/FSGS families and aim to identify new genomic loci for NS/..
  65. Jeffrey H Miner; Fiscal Year: 2016
    ..the role of genetic changes in the pathogenesis of glomerular diseases like focal segmental glomerulosclerosis (FSGS). In this application, we propose two specific aims...
  66. Altered gene expression using microarray in focal segmental glomerulosclerosis
    Kimberly Czech; Fiscal Year: 2009
    ..Lay summary: To better understand Focal segmental glomerulosclerosis (FSGS), a common type of kidney disease in adults and children, this project uses discarded frozen human kidney tissue ..
  67. CYTOCHROME P-450 AND GLOMERULAR PROTEIN PERMEABILITY
    Ellen T McCarthy; Fiscal Year: 2010
    Focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) are the most common causes of idiopathic nephrotic syndrome in humans...
  68. AUTOSOMAL DOMINANT FOCAL SEGMENTAL GLOMERULOSCLEROSIS
    Michelle Winn; Fiscal Year: 2004
    DESCRIPTION (adapted from the application) Focal segmental glomerulosclerosis (FSGS) is a pathological entity of unknown etiology characterized by proteinuria, nephrotic syndrome and the progressive loss of renal function...
  69. Polarized Targeting of Metabotropic Glutamate Receptors
    Anna Francesconi; Fiscal Year: 2004
    ..This application is ideally suited to the R21 format in that it employs high-risk experiments based on new technologies that may lead to major advances in this field. ..
  70. Modulation of beta1-integrin phosphorlyation by mutant alpha-actinin4 expression
    Michael Ross; Fiscal Year: 2009
    ..In our laboratory, mutations within the gene encoding alpha-actinin 4 (ACTN4) were identified as one of the genetic causes of inherited FSGS...
  71. Role of Gbeta 2 in the Nucleus of Angiotensin Receptor Activated Cells
    Anushree Bhatnagar; Fiscal Year: 2010
    ..Our proposed studies will demonstrate a novel mode of control of gene expression by a GPCR. ..