The mouse pink-eyed dilution gene: association with human Prader-Willi and Angelman syndromes

Science. 1992 Aug 21;257(5073):1121-4. doi: 10.1126/science.257.5073.1121.

Abstract

Complementary DNA clones from the pink-eyed dilution (p) locus of mouse chromosome 7 were isolated from murine melanoma and melanocyte libraries. The transcript from this gene is missing or altered in six independent mutant alleles of the p locus, suggesting that disruption of this gene results in the hypopigmentation phenotype that defines mutant p alleles. Characterization of the human homolog revealed that it is localized to human chromosome 15 at q11.2-q12, a region associated with Prader-Willi and Angelman syndromes, suggesting that altered expression of this gene may be responsible for the hypopigmentation phenotype exhibited by certain individuals with these disorders.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Carrier Proteins*
  • Chromosomes, Human, Pair 15
  • Cloning, Molecular
  • DNA / genetics
  • Humans
  • Melanocytes / chemistry
  • Melanoma, Experimental / chemistry
  • Membrane Proteins*
  • Membrane Transport Proteins*
  • Mice
  • Mice, Mutant Strains
  • Molecular Sequence Data
  • Mutation
  • Nucleic Acid Hybridization
  • Phenotype
  • Pigmentation Disorders / genetics*
  • Prader-Willi Syndrome / genetics*
  • Proteins / chemistry
  • Sequence Homology, Nucleic Acid

Substances

  • Carrier Proteins
  • Membrane Proteins
  • Membrane Transport Proteins
  • OCA2 protein, human
  • Proteins
  • P protein, mouse
  • DNA

Associated data

  • GENBANK/M97900
  • GENBANK/M97901