Spongiform Degeneration in mahoganoid Mutant Mice

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Science  31 Jan 2003:
Vol. 299, Issue 5607, pp. 710-712
DOI: 10.1126/science.1079694

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mahoganoid is a mouse coat-color mutation whose pigmentary phenotype and genetic interactions resemble those ofAttractin (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 inmahoganoid encodes a RING-containing protein with E3 ubiquitin ligase activity in vitro. Similarities in phenotype, expression, and genetic interactions suggest that mahoganoidand Atrn genes are part of a conserved pathway for regulated protein turnover whose function is essential for neuronal viability.

  • * To whom correspondence should be addressed. E-mail: gbarsh{at} (G.S.B.); tmg25{at}

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