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. 1994 Nov 1;13(21):5129–5134. doi: 10.1002/j.1460-2075.1994.tb06842.x

The glomerulosclerosis gene Mpv17 encodes a peroxisomal protein producing reactive oxygen species.

R M Zwacka 1, A Reuter 1, E Pfaff 1, J Moll 1, K Gorgas 1, M Karasawa 1, H Weiher 1
PMCID: PMC395460  PMID: 7957077

Abstract

The mutant mouse strain Mpv17 carries a retroviral insert in its genome which inactivates the Mpv17 gene. At a young age these mice develop glomerulosclerosis and nephrotic syndrome which resembles human disease. We show here that the Mpv17 gene product is highly conserved and encodes a peroxisomal protein. Loss of the Mpv17 protein does not impair peroxisome biogenesis but instead leads to a reduced ability to produce reactive oxygen species (ROS). In turn, overproduction of the Mpv17 gene in transfected cells results in dramatically enhanced levels of intracellular ROS indicating a direct involvement of Mpv17 in ROS production. These data reveal a role for the Mpv17 protein in peroxisomal reactive oxygen metabolism and establish a novel link between peroxisomal ROS production and glomerulosclerosis.

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