Morphologic Alterations and Biochemical Studies of the Glomerular Basement Membrane in Alport Syndrome

2015 ◽  
pp. 41-46 ◽  
Author(s):  
Gerald S. Spear
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Alport Syndrome ◽  
Biochemical Studies

scholarly journals Thin basement membrane disease – literature review

2015 ◽  
Vol 84 (3) ◽  
pp. 201-204
Author(s):  
Jakub Żurawski
Keyword(s):  
Electron Microscope ◽  
Basement Membrane ◽  
Literature Review ◽  
Iga Nephropathy ◽  
Glomerular Basement Membrane ◽  
Alport Syndrome ◽  
Clinical Course ◽  
Renal Diseases ◽  
Renal Lithiasis ◽  
Thin Basement Membrane Disease

Initially, the thin glomerular basement membrane disease was called “a gentle and curable hemorrhagic nephritis”. The thin basement membrane disease has been finally characterized at the beginning of 1970s. This is when the connection between previously clinically described gentle microhematuria and significant thinning of glomerular basement membrane discovered during examination under the electron-microscope has been established. Ultimately, the disease has been described as a condition characterized with a diverse clinical course, usually mild, but sometimes progressive. It is a family conditioned disease, but it also appears sporadically and concerns at least 1% of the population. It has also been stated that it is one of the most frequent renal diseases, enumerated directly after changes caused by infections, hypertension and renal lithiasis. This particular disease is diagnosed more often than IgA nephropathy and Alport syndrome, which are also associated with haematuria or microhematuria.

scholarly journals Albumin contributes to kidney disease progression in Alport syndrome

2016 ◽  
Vol 311 (1) ◽  
pp. F120-F130 ◽  
Author(s):  
George Jarad ◽  
Russell H. Knutsen ◽  
Robert P. Mecham ◽  
Jeffrey H. Miner
Keyword(s):  
Kidney Disease ◽  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Alport Syndrome ◽  
Transforming Growth Factor ◽  
Kidney Diseases ◽  
Serum Triglyceride ◽  
Transforming Growth Factor Β ◽  
Collagen Type Iv ◽  
Albumin Concentration

Alport syndrome is a familial kidney disease caused by defects in the collagen type IV network of the glomerular basement membrane. Lack of collagen-α3α4α5(IV) changes the glomerular basement membrane morphologically and functionally, rendering it leaky to albumin and other plasma proteins. Filtered albumin has been suggested to be a cause of the glomerular and tubular injuries observed at advanced stages of Alport syndrome. To directly investigate the role that albumin plays in the progression of disease in Alport syndrome, we generated albumin knockout ( Alb−/−) mice to use as a tool for removing albuminuria as a component of kidney disease. Mice lacking albumin were healthy and indistinguishable from control littermates, although they developed hypertriglyceridemia. Dyslipidemia was observed in Alb+/− mice, which displayed half the normal plasma albumin concentration. Alb mutant mice were bred to collagen-α3(IV) knockout ( Col4a3−/−) mice, which are a model for human Alport syndrome. Lack of circulating and filtered albumin in Col4a3−/−; Alb−/− mice resulted in dramatically improved kidney disease outcomes, as these mice lived 64% longer than did Col4a3−/−; Alb+/+ and Col4a3−/−; Alb+/− mice, despite similar blood pressures and serum triglyceride levels. Further investigations showed that the absence of albumin correlated with reduced transforming growth factor-β1 signaling as well as reduced tubulointerstitial, glomerular, and podocyte pathology. We conclude that filtered albumin is injurious to kidney cells in Alport syndrome and perhaps in other proteinuric kidney diseases, including diabetic nephropathy.

scholarly journals The Accumulation of VEGFA in the Glomerular Basement Membrane and Its Relationship with Podocyte Injury and Proteinuria in Alport Syndrome

PLoS ONE ◽  
2015 ◽  
Vol 10 (8) ◽  
pp. e0135648 ◽  
Author(s):  
Haiyan Wang ◽  
Zhihui Yue ◽  
Jinlang Wu ◽  
Ting Liu ◽  
Ying Mo ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Alport Syndrome ◽  
Podocyte Injury

scholarly journals Alport syndrome and Pierson syndrome: Diseases of the glomerular basement membrane

2018 ◽  
Vol 71-72 ◽  
pp. 250-261 ◽  
Author(s):  
Steven D. Funk ◽  
Meei-Hua Lin ◽  
Jeffrey H. Miner
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Alport Syndrome ◽  
Pierson Syndrome
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