Spongiform degeneration in mahoganoid mutant mice.

  title={Spongiform degeneration in mahoganoid mutant mice.},
  author={Lin He and Xin-Yun Lu and Aaron F Jolly and Adam G. Eldridge and Stanley J. Watson and Peter Kent Jackson and Gregory S Barsh and Teresa M. Gunn},
  volume={299 5607},
mahoganoid is a mouse coat-color mutation whose pigmentary phenotype and genetic interactions resemble those of Attractin (Atrn). Atrn mutations also cause spongiform neurodegeneration. Here, we show that a null mutation for mahoganoid causes a similar age-dependent neuropathology that includes many features of prion diseases but without accumulation of protease-resistant prion protein. The gene mutated in mahoganoid encodes a RING-containing protein with E3 ubiquitin ligase activity in vitro… CONTINUE READING

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