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| ALPORTS Hepinstall's Pathology of the Kidney, 6th Edition |
Alport syndrome is a genetic defect of type 4 collagen, a major structural component of the glomerular basement membrane. Which is in turn the major structural component that defines the function of the filtration apparatus of the kidney. Defects of this important component of glomerular function results in various abnormalities. In Alport syndrome patients progressively lose renal function due to inflammation of the kidney. There is currently no known cure for this condition.
Researchers in Boston however have published a paper that seeks to lay the foundation of a possible future treatment. Their research focuses on a lab induced variant of Alport syndrome in rats and its subsequent treatment with stem cells derived from the bone marrow of healthy rats. The results so far suggest that the damage caused by the mutant type of collagen in Alport syndrome is repaired by infusion of both bone marrow derived stem cells from rats as well as human stem cells.
The findings are fairly exciting because there was benefit to be had even in advanced disease. The missing collagen being synthesized and introduced into the abnormal basement membranes subsequently resulting in reorganization of the structural components of the basement membrane and improved kidney function.
The technical aspects have yet to be worked out sufficiently to even provide a best guess timeline for possible human trials but the ground is fruitful for further research.
Where is the renal stem cell indeed?
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