The Wsh and Ph mutations affect the c-kit expression profile: c-kit misexpression in embryogenesis impairs melanogenesis in Wsh and Ph mutant mice

Proc Natl Acad Sci U S A. 1995 Apr 25;92(9):3754-8. doi: 10.1073/pnas.92.9.3754.

Abstract

The receptor tyrosine kinases (RTKs) c-kit and platelet-derived growth factor receptor alpha chain (PDG-FRa) are encoded at the white spotting (W) and patch (Ph) loci on mouse chromosome 5. While W mutations affect melanogenesis, gametogenesis, and hematopoiesis, the Ph mutation affects melanogenesis and causes early lethality in homozygotes. W-sash (Wsh) is an expression mutation and blocks c-kit expression in certain cell types and enhances c-kit expression in others, including at sites important for early melanogenesis. We have determined the effect of Ph on c-kit expression during embryogenesis in Ph heterozygotes. Immunohistochemical analysis revealed enhanced c-kit expression in several cell types, including sites important for early melanogenesis. We propose that in both Wsh and Ph mutant mice c-kit misexpression affects early melanogenesis and is responsible for the pigment deficiency. Moreover, we have defined the organization of the RTKs in the W/Ph region on chromosome 5 and characterized the Wsh mutation by using pulsed-field gel electrophoresis. Whereas the order of the RTK genes was determined as Pdgfra-c-kit-flk1, analysis of the Wsh mutation revealed that the c-kit and Pdgfra genes are unlinked in Wsh, presumably because of an inversion of a small segment of chromosome 5. The Ph mutation consists of a deletion including Pdgfra and the 3' deletion endpoint of Ph lies between Pdgfra and c-kit. Therefore, positive 5' upstream elements controlling c-kit expression in mast cells and some other cell types are affected by the Wsh mutation and negative elements are affected by both the Wsh and the Ph mutation.

Publication types

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

MeSH terms

  • Animals
  • Base Sequence
  • Chromosome Mapping*
  • DNA Primers
  • Embryo, Mammalian / physiology*
  • Gene Expression*
  • Immunohistochemistry
  • Mice
  • Mice, Inbred C57BL / genetics*
  • Molecular Sequence Data
  • Mutation*
  • Polymerase Chain Reaction
  • Proto-Oncogene Proteins / analysis
  • Proto-Oncogene Proteins / biosynthesis*
  • Proto-Oncogene Proteins / genetics
  • Proto-Oncogene Proteins c-kit
  • Proto-Oncogenes*
  • Receptor Protein-Tyrosine Kinases / analysis
  • Receptor Protein-Tyrosine Kinases / biosynthesis*
  • Receptor Protein-Tyrosine Kinases / genetics
  • Receptors, Colony-Stimulating Factor / analysis
  • Receptors, Colony-Stimulating Factor / biosynthesis*
  • Receptors, Colony-Stimulating Factor / genetics
  • Receptors, Platelet-Derived Growth Factor / biosynthesis
  • Receptors, Platelet-Derived Growth Factor / genetics*
  • Restriction Mapping

Substances

  • DNA Primers
  • Proto-Oncogene Proteins
  • Receptors, Colony-Stimulating Factor
  • Proto-Oncogene Proteins c-kit
  • Receptor Protein-Tyrosine Kinases
  • Receptors, Platelet-Derived Growth Factor