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Spongiform degeneration in mahoganoid mutant mice.

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.

Pubmed ID: 12560552


  • He L
  • Lu XY
  • Jolly AF
  • Eldridge AG
  • Watson SJ
  • Jackson PK
  • Barsh GS
  • Gunn TM


Science (New York, N.Y.)

Publication Data

January 31, 2003

Associated Grants


Mesh Terms

  • Alleles
  • Amino Acid Sequence
  • Animals
  • Blotting, Northern
  • Brain
  • Carrier Proteins
  • Crosses, Genetic
  • Female
  • Gene Expression
  • Ligases
  • Male
  • Membrane Proteins
  • Mice
  • Mice, Inbred C3H
  • Mice, Mutant Strains
  • Mice, Transgenic
  • Models, Biological
  • Molecular Sequence Data
  • Mutation
  • Neurodegenerative Diseases
  • Neurons
  • Pigmentation
  • Prions
  • RNA, Messenger
  • Recombinant Fusion Proteins
  • Transgenes
  • Ubiquitin
  • Ubiquitin-Protein Ligases
  • Vacuoles