Identification of one novel mutation in the C-propeptide of COL2A1 in a Chinese family with spondyloperipheral dysplasia

Gene. 2013 Jun 10;522(1):107-10. doi: 10.1016/j.gene.2013.03.083. Epub 2013 Mar 29.

Abstract

Spondyloperipheral dysplasia (SPD; OMIM 271700) is an autosomal dominant connective tissue disorder characterized by vertebral body abnormalities (platyspondyly, end-plate indentations), hip dysplasia and brachydactyly type E. Here, we identified a novel truncating mutation (p.Lys1444AsnfsX27) in the C-propeptide of type II collagen in an affected Chinese individual with SPD. Our findings will provide clues to the phenotype-genotype relations and may assist not only in the clinical diagnosis of SPD but also in the interpretation of genetic information used for prenatal diagnosis and genetic counseling.

Publication types

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

MeSH terms

  • Adult
  • Asian People
  • Collagen Type II / genetics*
  • Female
  • Genetic Predisposition to Disease
  • Genotype
  • Humans
  • Mutation*
  • Osteochondrodysplasias / genetics*

Substances

  • COL2A1 protein, human
  • Collagen Type II

Supplementary concepts

  • Spondyloperipheral dysplasia short ulna