Novel RDH5 mutation in family with mother having fundus albipunctatus and three children with retinitis pigmentosa

Ophthalmic Genet. 2008 Mar;29(1):29-32. doi: 10.1080/13816810701663535.

Abstract

Purpose: To identify mutations in the RDH5 gene in a family with a mother having fundus albipunctatus (FA) and 3 children with retinitis pigmentosa (RP).

Methods: Ophthalmological examinations were performed to diagnose FA and RP. Mutational analysis of RDH5 was performed.

Results/conclusions: The mother was diagnosed with FA, and 3 children were diagnosed with RP. The proband's mother, brother, and sister had a novel mutation c.689_690CT > GG in RDH5. The proband and mother had a previously reported mutation c.928delCinsGAAG. Consequently, the mother's FA was caused by compound heterozygous mutations. Further studies will be needed to determine the gene responsible for children's RP.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Alcohol Oxidoreductases / genetics*
  • Child
  • Female
  • Fundus Oculi
  • Genes, Recessive*
  • Heterozygote
  • Humans
  • Mutation, Missense
  • Night Blindness / genetics*
  • Night Blindness / pathology
  • Pedigree
  • Retina / pathology
  • Retinitis Pigmentosa / genetics*

Substances

  • Alcohol Oxidoreductases
  • retinol dehydrogenase 5