Focal and segmental glomerulosclerosis: varying biologic mechanisms underlie a final histopathologic end point.
Focal and segmental glomerulosclerosis (FSGS) is a pathologic entity that is a common and increasing cause of end-stage renal disease. Typical manifestations include proteinuria, hypertension, worsening renal insufficiency, and, frequently, renal failure. The etiology, however, remains unknown in a majority of patients. There is an estimated recurrence rate of 30% to 40% in renal transplant patients, suggesting that the pathogenesis is not solely a result of intrinsic kidney disease. Although some of its characteristics have been reported, the precise identification of a circulating factor associated with FSGS has not been made. Remarkable progress has been made in recent years regarding biologic mechanisms surrounding FSGS and proteinuria. Insight into the pathogenesis of FSGS has been gained through the study of hereditary forms of FSGS and nephrotic syndromes. Mutations in cytoskeletal proteins that affect podocyte structure have been the target until recently. Here we review the current understanding of this glomerular disease and areas for future concentration.
Daskalakis
Nikki
N
Department of Medicine, and the Center for Human Genetics, Duke University Medical Center, Durham, NC 27710, USA.
Winn
Michelle P
MP
D048868:Adaptor Proteins, Signal Transducing; D015415:Biomarkers / Q000097:blood*; D003598:Cytoskeletal Proteins / Q000097:blood; D018450:Disease Progression; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000378:metabolism / Q000473:pathology; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000473:pathology; D008565:Membrane Proteins / Q000097:blood; D012307:Risk Factors; D050052:TRPC Cation Channels / Q000097:blood; D000074287:TRPC6 Cation Channel; D018909:src Homology Domains
D048868:Adaptor Proteins, Signal Transducing; D015415:Biomarkers; C114545:CD2-associated protein; D003598:Cytoskeletal Proteins; D047908:Intracellular Signaling Peptides and Proteins; D008565:Membrane Proteins; C407223:NPHS2 protein; D050052:TRPC Cation Channels; D000074287:TRPC6 Cation Channel; C117032:TRPC6 protein, human; C113267:nephrin