First Author | Zwacka RM | Year | 1994 |
Journal | EMBO J | Volume | 13 |
Issue | 21 | Pages | 5129-34 |
PubMed ID | 7957077 | Mgi Jnum | J:21477 |
Mgi Id | MGI:69439 | Doi | 10.1002/j.1460-2075.1994.tb06842.x |
Citation | Zwacka RM, et al. (1994) The glomerulosclerosis gene Mpv17 encodes a peroxisomal protein producing reactive oxygen species. EMBO J 13(21):5129-34 |
abstractText | 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. |