[Genotype-phenotype correlation in Chinese patients with retinitis pigmentosa due to rhodopsin mutation]

Yan Ke Xue Bao. 1999 Dec;15(4):204-6, 235.
[Article in Chinese]

Abstract

Purpose: To investigate the genotype-phenotype correlation in Chinese patients with retinitis pigmentosa caused by rhodopsin gene mutation.

Methods: On the basis of the onset of symptoms, degree of morphological changes and progression of visual disability in three (3/83) patients with identified mutations, the correlation of the phenotype with the corresponding mutations was assessed.

Results: There was a certain degree of allele-specificity. Severe form of retinitis pigmentosa was found in patients with mutation in the cytoplasmic domain and mild form of retinitis pigmentosa in patients with mutation in the intradiscal domain.

Conclusion: Although there is a certain relation between the mutant rhodopsin and ocular manifestation, we need to accumulate more materials before relating a rhodopsin mutation to a specific phenotype.

Publication types

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

MeSH terms

  • Adult
  • Alleles
  • Asian People
  • Female
  • Genes, Dominant
  • Genotype
  • Humans
  • Male
  • Pedigree
  • Phenotype
  • Point Mutation*
  • Retinitis Pigmentosa / genetics*
  • Rhodopsin / genetics*
  • Visual Acuity

Substances

  • Rhodopsin