Novel SHOX gene mutation in a short boy with Becker muscular dystrophy: double trouble in two adjacent genes

Horm Res. 2008;69(2):124-8. doi: 10.1159/000111816. Epub 2007 Dec 5.

Abstract

Short stature is a well-recognized feature of Duchenne muscular dystrophy, whilst it has been reported rarely in Becker muscular dystrophy (BMD). Here we report two brothers with BMD, who exhibited a very different growth pattern. Whereas in the short brother (-2.2 SDS) molecular investigation revealed a G367A mutation in the short stature homeobox containing (SHOX) gene located in the Xp22.3 region, no abnormality was found in the brother with normal height (-0.1 SDS). Our data suggest that abnormal growth observed in a boy with BMD may be related to an additional genetic alteration, already known as correlated with short stature.

Publication types

  • Case Reports

MeSH terms

  • Base Sequence
  • Child
  • Child, Preschool
  • DNA Mutational Analysis
  • Growth Disorders / genetics*
  • Homeodomain Proteins / genetics*
  • Humans
  • Muscular Dystrophy, Duchenne / genetics*
  • Mutation
  • Short Stature Homeobox Protein

Substances

  • Homeodomain Proteins
  • SHOX protein, human
  • Short Stature Homeobox Protein