Rhizomelic chondrodysplasia punctata type 1: report of mutations in 3 children from India

J Appl Genet. 2010;51(1):107-10. doi: 10.1007/BF03195717.

Abstract

Rhizomelic chondrodysplasia punctata is a rare autosomal recessive disorder characterized by stippled epiphyses and rhizomelic shortening of the long bones. We report 3 subjects of rhizomelic chondrodysplasia punctata from India and the PEX7 mutations identified in them. The common PEX7-L292X allele, whose high frequency is due to a founder effect in the northern European Caucasian population, was not identified in these patients. Instead, 2 novel alleles are described, including 64_65delGC, which was present on a single PEX7 haplotype and could represent a common allele in the Indian population.

Publication types

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

MeSH terms

  • Adolescent
  • Chondrodysplasia Punctata, Rhizomelic / genetics*
  • Chondrodysplasia Punctata, Rhizomelic / pathology*
  • Female
  • Founder Effect
  • Humans
  • India
  • Infant
  • Infant, Newborn
  • Male
  • Mutation / genetics*
  • Peroxisomal Targeting Signal 2 Receptor
  • Receptors, Cytoplasmic and Nuclear / genetics*

Substances

  • PEX7 protein, human
  • Peroxisomal Targeting Signal 2 Receptor
  • Receptors, Cytoplasmic and Nuclear