Gene deletions in Japanese patients with Duchenne and Becker muscular dystrophies: deletion study and carrier detection

Clin Genet. 1991 Jun;39(6):419-24. doi: 10.1111/j.1399-0004.1991.tb03052.x.

Abstract

Fifty unrelated Japanese patients with Duchenne and Becker muscular dystrophy (DMD and BMD) have been studied through use of the dystrophin cDNA probes. The 14-kb dystrophin cDNA was subdivided into six subclones, and Hind III-digested DNAs were analyzed by Southern blotting. Of 50 unrelated patients, 20 showed a deletion of one or several of the exon-containing Hind III fragments (40.0%). These corresponded to 50% (11/22) of BMD patients and 32.1% (9/28) of DMD patients, and the position and extent of deletions were mapped and proven to be more heterogeneous in DMD than in BMD. Both ends of deletions detected by probe 1-2a were common to all six BMD patients, and the 5' ends of deletions in probe 5b-7 were also common to four BMD patients. The phenotypic-specific deletion in Japanese BMD patients existed in the 5' end of the DMD gene, although an apparently similar deletion produced a wide range of clinical courses (BMD phenotype). Three out of eight females in DMD/BMD families were diagnosed as carriers through use of the junctional fragment and dosage analyses of dystrophin cDNA.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Blotting, Southern
  • Child
  • Chromosome Deletion*
  • Chromosome Mapping
  • DNA Probes
  • Deoxyribonuclease HindIII / genetics
  • Dosage Compensation, Genetic
  • Dystrophin
  • Female
  • Genetic Carrier Screening*
  • Humans
  • Japan
  • Male
  • Muscular Dystrophies / classification
  • Muscular Dystrophies / epidemiology
  • Muscular Dystrophies / genetics*
  • Pedigree
  • Phenotype

Substances

  • DNA Probes
  • Dystrophin
  • Deoxyribonuclease HindIII