Article
Translation inhibition corrects aberrant localization of mutant alanine-glyoxylate aminotransferase: possible therapeutic approach for hyperoxaluria.
Journal of molecular medicine (Berlin, Germany) - 1 Jul 2018
Belostotsky Ruth, Lyakhovetsky Roman, Sherman Michael Y, Shkedy Fanny, Tzvi-Behr Shimrit, Bar Roi, Hoppe Bernd, Reusch Björn, Beck Bodo B, Frishberg Yaacov
Abstract excerpt
Primary hyperoxaluria type 1 is a severe kidney stone disease caused by abnormalities of the peroxisomal alanine-glyoxylate aminotransferase (AGT). The most frequent mutation G170R results in aberrant mitochondrial localization of the active enzyme. To evaluate the population of peroxisome-localized AGT, we developed a quantitative Glow-AGT assay based on the self-assembly split-GFP approach and used it to...
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