Exclusion of RAI2 as the causative gene for Nance-Horan syndrome

Hum Genet. 1999 May;104(5):410-1. doi: 10.1007/s004390050976.

Abstract

Nance-Horan syndrome (NHS) is an X-linked condition characterised by congenital cataracts, microphthalmia and/or microcornea, unusual dental morphology, dysmorphic facial features, and developmental delay in some cases. Recent linkage studies have mapped the NHS disease gene to a 3.5-cM interval on Xp22.2 between DXS1053 and DXS443. We previously identified a human homologue of a mouse retinoic-acid-induced gene (RAI2) within the NHS critical flanking interval and have tested the gene as a candidate for Nance-Horan syndrome in nine NHS-affected families. Direct sequencing of the RAI2 gene and predicted promoter region has revealed no mutations in the families screened; RAI2 is therefore unlikely to be associated with NHS.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple / genetics*
  • Animals
  • Base Sequence
  • Cataract / congenital
  • Cataract / genetics
  • Chromosome Mapping
  • Cornea / abnormalities
  • DNA / blood
  • Face / abnormalities
  • Humans
  • Intellectual Disability / genetics
  • Intercellular Signaling Peptides and Proteins
  • Lymphocytes / pathology
  • Mice
  • Microphthalmos / genetics
  • Promoter Regions, Genetic
  • Proteins / genetics*
  • Syndrome
  • X Chromosome*

Substances

  • Intercellular Signaling Peptides and Proteins
  • Proteins
  • RAI2 protein, human
  • DNA