Prenatal diagnosis of muscle-eye-brain disease

Prenat Diagn. 2007 Jan;27(1):51-4. doi: 10.1002/pd.1622.

Abstract

Objectives: To present a family in which it was possible to perform prenatal diagnosis for the recessively inherited muscle-eye-brain disease (MEB) using linkage analysis.

Methods: Linkage analysis and direct sequencing of the POMGNT1 gene were carried out in a Turkish MEB family with one affected individual. Fetal DNA was obtained from an ongoing pregnancy by chorionic villus sampling (CVS).

Results: Both linkage analysis of the POMGNT1/1p32-p34 region and direct sequencing for the novel familial mutation (R605H) demonstrated that the fetus did not have MEB.

Conclusion: We report the first case of prenatal diagnosis in MEB by molecular genetic analysis.

MeSH terms

  • Adolescent
  • Brain Diseases / diagnosis*
  • Brain Diseases / genetics
  • Chorionic Villi Sampling
  • Chromosome Mapping
  • Consanguinity
  • Eye Diseases, Hereditary / diagnosis*
  • Eye Diseases, Hereditary / genetics
  • Female
  • Fetal Diseases / genetics
  • Humans
  • Magnetic Resonance Imaging
  • Muscular Dystrophies / diagnosis*
  • Muscular Dystrophies / genetics
  • N-Acetylglucosaminyltransferases / genetics*
  • Pedigree
  • Pregnancy
  • Prenatal Diagnosis*

Substances

  • N-Acetylglucosaminyltransferases
  • protein O-mannose beta-1,2-N-acetylglucosaminyltransferase