Spongiform degeneration in mahoganoid mutant mice

Lin He, Xin Yun Lu, Aaron F. Jolly, Adam G. Eldridge, Stanley J. Watson, Peter K. Jackson, Gregory S. Barsh, Teresa M. Gunn

Research output: Contribution to journalArticlepeer-review

119 Scopus citations


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. Similarities in phenotype, expression, and genetic interactions suggest that mahoganoid and Atrn genes are part of a conserved pathway for regulated protein turnover whose function is essential for neuronal viability.

Original languageEnglish (US)
Pages (from-to)710-712
Number of pages3
Issue number5607
StatePublished - Jan 31 2003

ASJC Scopus subject areas

  • General


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