The glomerulosclerosis gene Mpv 17 encodes a peroxisomal protein producing reactive oxygenspecies

Zwacka, R. M.; Reuter, A.; Pfaff, Eberhard GND; Moll, J.; Gorgas, K.; Karasawa, M; Weiher, H.

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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Zwacka, R. / Reuter, A. / Pfaff, Eberhard / et al: The glomerulosclerosis gene Mpv 17 encodes a peroxisomal protein producing reactive oxygenspecies. 1994.

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