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

EMBO J. 1994 Nov 1;13(21):5129-34. doi: 10.1002/j.1460-2075.1994.tb06842.x.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Catalase / isolation & purification
  • Cell Compartmentation
  • Disease Models, Animal
  • Fibroblasts / ultrastructure
  • Fluorescent Antibody Technique
  • Glomerulonephritis / genetics*
  • Liver / ultrastructure
  • Membrane Proteins*
  • Mice
  • Mice, Mutant Strains
  • Microbodies / metabolism*
  • Molecular Sequence Data
  • Nephrotic Syndrome / genetics*
  • Proteins / genetics*
  • Proteins / isolation & purification
  • Proteins / metabolism
  • Reactive Oxygen Species / metabolism*
  • Sequence Homology, Amino Acid


  • Membrane Proteins
  • Mpv17 protein, mouse
  • Proteins
  • Reactive Oxygen Species
  • Catalase