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EMBO J. 1994 Nov 1;13(21):5129-34.

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

Author information

  • 1Kernforschungszentrum Karlsruhe, Institut für Genetik, Karlsruhe, Germany.

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.

PMID:
7957077
[PubMed - indexed for MEDLINE]
PMCID:
PMC395460
Free PMC Article
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