Paradominant inheritance, a hypothesis explaining occasional familial occurrence of sporadic syndromes

Am J Med Genet. 1999 Aug 6;85(4):359-60. doi: 10.1002/(sici)1096-8628(19990806)85:4<359::aid-ajmg10>3.0.co;2-v.

Abstract

Heterozygous individuals carrying a "paradominant" mutation, as a rule, are phenotypically normal. Therefore, the mutation can be transmitted unperceived through many generations. The trait becomes manifest when a somatic mutation occurs during embryogenesis giving rise to loss of heterozygosity and forming a mutant cell population, being either homozygous or hemizygous for the mutation. This concept explains the occasional familial occurrence of usually sporadic traits like the Klippel-Trenaunay syndrome and others.

Publication types

  • Review

MeSH terms

  • Genes, Dominant*
  • Humans
  • Klippel-Trenaunay-Weber Syndrome / genetics
  • Nevus / complications
  • Nevus / genetics*
  • Skin Neoplasms / complications
  • Skin Neoplasms / genetics*
  • Syndrome