| 17527 |
P19258 |
MPV17_MOUSE |
Protein Mpv17 (Mpv-17) |
DISRUPTION PHENOTYPE |
Mice lack expression of this protein resulting in the development of adult onset nephrotic syndrome and chronic renal failure. They also develop severe morphological degeneration of the inner ear. In the inner ear, mice lacking Mpv17 display degenerative changes of the cochlear structures already at the age of 2 months. The degenerative changes are patchy arranged throughout the entire length of the cochlea and involved the organ of Corti as well as the stria vascularis epithelia with alterations of the basement membrane of the capillaries. {ECO:0000269|PubMed:11377883, ECO:0000269|PubMed:9447939}. |