• Media type: E-Article
  • Title: Camurati–engelmann disease with obesity in a newly identified family carrying a missense p.Arg156Cys mutation in the TGFB1 gene
  • Contributor: Collet, Corinne; Laplanche, Jean‐Louis; de Vernejoul, Marie‐Christine
  • Published: Wiley, 2013
  • Published in: American Journal of Medical Genetics Part A, 161 (2013) 8, Seite 2074-2077
  • Language: English
  • DOI: 10.1002/ajmg.a.36022
  • ISSN: 1552-4825; 1552-4833
  • Keywords: Genetics (clinical) ; Genetics
  • Origination:
  • Footnote:
  • Description: AbstractWe report on a family affected by Camurati–Engelmann disease, characterized by radiological signs limited to the tibia, and associated with overweight or obesity, which is not a known feature of this disorder. The affected patients were heterozygous for a c.466C > T mutation (which predicts p.Arg156Cys) in the latency associated protein (LAP)‐coding domain of the TGFB1 gene. This mutation had previously been reported once in another family with a similar, atypical phenotype, which suggests a possible phenotype/genotype relationship. © 2013 Wiley Periodicals, Inc.