Failure in detecting mRNA transcripts from the mutated allele in myotonic dystrophy muscle

Biochem Mol Biol Int. 1993 Feb;29(2):291-7.

Abstract

The expression of myotonin-protein kinase (MT-PK) gene was studied in myotonic dystrophy (DM) muscle and normal controls using a polymerase chain reaction protocol to analyse the 3' intragenic p(CTG) polymorphism. Unaffected individuals show bi-allelic expression, while the sole wild-type allele was transcribed in DM muscle. Our findings support a gene dosage effect in the pathogenesis of the disease.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Alleles
  • Blotting, Southern
  • DNA / biosynthesis
  • Electrophoresis, Polyacrylamide Gel
  • Fetus / metabolism
  • Gene Expression Regulation, Enzymologic*
  • Humans
  • Muscles / enzymology*
  • Mutation
  • Myotonic Dystrophy / enzymology
  • Myotonic Dystrophy / genetics*
  • Myotonin-Protein Kinase
  • Polymerase Chain Reaction
  • Polymorphism, Genetic
  • Protein Kinases / chemistry
  • Protein Kinases / genetics*
  • Protein Serine-Threonine Kinases*
  • RNA, Messenger / analysis
  • RNA, Messenger / genetics*
  • Transcription, Genetic

Substances

  • DMPK protein, human
  • RNA, Messenger
  • DNA
  • Protein Kinases
  • Myotonin-Protein Kinase
  • Protein Serine-Threonine Kinases

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