MYO1E, Focal Segmental Glomerulosclerosis, and the Cytoskeleton

Focal segmental glomerulosclerosis, one of the most common glomerulopathies, is typically heralded by massive proteinuria, often associated with unremitting nephrotic syndrome and inexorable progression to end-stage kidney disease. 1 – 3 Unfortunately, therapy is frequently unsuccessful — many patie...

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Published inThe New England journal of medicine Vol. 365; no. 4; pp. 368 - 369
Main Author Ingelfinger, Julie R
Format Journal Article
LanguageEnglish
Published United States Massachusetts Medical Society 28.07.2011
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Abstract Focal segmental glomerulosclerosis, one of the most common glomerulopathies, is typically heralded by massive proteinuria, often associated with unremitting nephrotic syndrome and inexorable progression to end-stage kidney disease. 1 – 3 Unfortunately, therapy is frequently unsuccessful — many patients with this disorder do not respond to treatment with glucocorticoids, and immunosuppressive therapy often fails as well. The disease also frequently recurs after kidney transplantation. The podocyte is critical to the development of focal segmental glomerulosclerosis. This dynamic cell controls glomerular filtration by means of its slit-pore diaphragm and other properties, which include motility along the glomerular basement membrane. 4 Podocytes surround and adhere . . .
AbstractList Focal segmental glomerulosclerosis, one of the most common glomerulopathies, is typically heralded by massive proteinuria, often associated with unremitting nephrotic syndrome and inexorable progression to end-stage kidney disease. 1 – 3 Unfortunately, therapy is frequently unsuccessful — many patients with this disorder do not respond to treatment with glucocorticoids, and immunosuppressive therapy often fails as well. The disease also frequently recurs after kidney transplantation. The podocyte is critical to the development of focal segmental glomerulosclerosis. This dynamic cell controls glomerular filtration by means of its slit-pore diaphragm and other properties, which include motility along the glomerular basement membrane. 4 Podocytes surround and adhere . . .
Focal segmental glomerulosclerosis, one of the most common glomerulopathies, is typically heralded by massive proteinuria, often associated with unremitting nephrotic syndrome and inexorable progression to end-stage kidney disease.1–3 Unfortunately, therapy is frequently unsuccessful — many patients with this disorder do not respond to treatment with glucocorticoids, and immunosuppressive therapy often fails as well. The disease also frequently recurs after kidney transplantation. The podocyte is critical to the development of focal segmental glomerulosclerosis. This dynamic cell controls glomerular filtration by means of its slit-pore diaphragm and other properties, which include motility along the glomerular basement membrane.4 Podocytes surround and adhere . . .
Author Ingelfinger, Julie R
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CitedBy_id crossref_primary_10_1038_nrneph_2012_19
crossref_primary_10_1155_2012_314251
crossref_primary_10_1101_gr_155697_113
crossref_primary_10_1371_journal_pone_0072750
Cites_doi 10.1056/NEJMoa1101273
10.1007/s00467-010-1692-x
10.1038/nm.1857
10.1111/j.1523-1755.2005.00723.x
10.1093/ndt/gfq617
10.1053/j.ajkd.2003.10.024
10.1093/ndt/gfg1045
10.1126/scitranslmed.3002231
10.1007/s00431-009-1017-x
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Snippet Focal segmental glomerulosclerosis, one of the most common glomerulopathies, is typically heralded by massive proteinuria, often associated with unremitting...
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SubjectTerms Calcineurin Inhibitors
Cyclosporine - pharmacology
Cyclosporine - therapeutic use
Cytoskeleton
Cytoskeleton - drug effects
Diaphragm
Disease
Drug Resistance
End-stage renal disease
Enzyme Inhibitors - pharmacology
Enzyme Inhibitors - therapeutic use
Genes
Glomerulosclerosis, Focal Segmental - drug therapy
Glomerulosclerosis, Focal Segmental - genetics
Glucocorticoids
Glucocorticoids - pharmacology
Glucocorticoids - therapeutic use
Humans
Immunosuppressive agents
Kidney diseases
Kidney transplantation
Motility
Mutation
Myosin Type I - genetics
Nephrotic syndrome
Podocytes - drug effects
Podocytes - ultrastructure
Proteins
Proteinuria
Title MYO1E, Focal Segmental Glomerulosclerosis, and the Cytoskeleton
URI http://dx.doi.org/10.1056/NEJMe1106093
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