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Inherited Diseases of the Glomerular Basement Membrane
American Journal of Kidney Diseases, 1994The inherited diseases of the glomerular basement membrane include Alport's syndrome (AS), nail-patella syndrome, and thin basement membrane nephropathy. Classical AS is inherited in an X-linked manner and accounts for approximately 85% of the cases. Its manifestations include hematuria, sensorineural hearing loss, ocular defects, and a progression to ...
William S. Hammond +3 more
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Molecular Sieving by Glomerular Basement Membrane
Nature, 1967OF the three layers of the glomerular capillary wall—endothelial cells, basement membrane and epithelial cells—the basement membrane appears to be the only complete barrier between the plasma and the glomerular filtrate1,2. Support for the view that the basement membrane is the most important filtering mechanism was provided by the studies on ferritin ...
Linda Hutton +5 more
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Pathology of glomerular basement membrane nephropathy
Current Opinion in Nephrology and Hypertension, 1994Studies of the molecular pathology of glomerular basement membrane have led to the discovery of four novel chains of type IV collagen, the latest of which is the alpha 6(IV) chain. Three chains have been shown to be directly involved in the pathogenesis of Goodpasture syndrome, Alport syndrome, and Alport posttransplantation antiglomerular basement ...
Raghuram Kalluri +2 more
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Immunogenetics of the Glomerular Basement Membrane
1990The nature of the genetic defect responsible for the abnormalities of kidney, cochlea, and eye that are characteristic of Alport’s syndrome has been a matter of interest to nephrologists, geneticists, and pathologists for decades. In recent years this entity has been the subject of intensive laboratory research, the results of which indicate that the ...
Clifford E. Kashtan, Alfred F. Michael
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Inherited diseases of the glomerular basement membrane [PDF]
Nature Clinical Practice Nephrology, 2008 The glomerular basement membrane (GBM) is a specialized form of basement membrane that has a major role in the maintenance of the glomerular filtration barrier. Like all basement membranes, it contains four main components: type IV collagen, laminin, nidogen, and heparan sulfate proteoglycans.
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The Glomerular Basement Membrane
1981The basement membrane of the glomerular capillaries of the mammalian kidney has been a favorite object for studies on the structure, function, and composition of basement membranes because of its important biological role in glomerular filtration of macromolecules, and because of the relative ease with which it can be isolated for study, free from ...
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Glomerular Basement Membrane Antigens
1983In experimental nephritis produced by the administration of nephrotoxic serum, there is in vivo localization of heterologous immunoglobulin antibodies in a smooth linear pattern along the glomerular basement membrane (GBM) (Hammer and Dixon, 1963). Nephrotoxic serum, produced by immunization with isolated GBM, reacts in vitro by fixing not only to GBM,
Mark S. Schiffer +3 more
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Genetic Disorders of Glomerular Basement Membranes
Nephron Clinical Practice, 2010This review provides current information about glomerular disorders that arise directly from inherited abnormalities in extracellular matrix proteins intrinsic to the glomerular basement membrane (Alport syndrome, thin basement membrane nephropathy, HANAC syndrome and Pierson syndrome).
Clifford E. Kashtan, Yoav Segal
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The glomerular basement membrane in lupus nephritis
Microscopy Research and Technique, 1994AbstractSystemic lupus erythematosus is a disease that is particularly suited for studies of glomerular basement membrane pathology. Classification of the renal pathology of lupus nephritis is usually based on light microscopic features, combined with immunofluorescence findings and electron microscopic alterations.
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Glomerular Podocytes and Adhesive Interaction with Glomerular Basement Membrane
Nephron Experimental Nephrology, 1999Normal podocyte function requires attachment to the underlying glomerular basement membrane. Alteration or disruption of podocyte attachment occurs in many forms of glomerular injury, leading to the development of proteinuria and eventually progressive glomerulosclerosis.
Peter Mundel, Stuart J. Shankland
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