Brain- and muscle-type promoters of the dystrophin gene are selected in peripheral lymphocytes and Epstein Barr virus-transformed lymphoblastoid [correction of lymphoplastoid] cells

J Neurol. 1993 Dec;241(2):81-6. doi: 10.1007/BF00869768.

Abstract

Promoter-specific transcripts of the dystrophin gene in peripheral lymphocytes and Epstein Barr virus-transformed lymphoblastoid cells were analysed using reverse transcription-nested polymerase chain reaction. Two DNA fragments, corresponding to the alternative first exons transcribed from either brain- or muscle-type promoters, were both amplified from cDNA prepared from normal lymphocytes and lymphoblastoid cells. The nucleotide sequences of the amplified products were 100% homologous to the 5' termini of the cDNA of brain- and muscle-type dystrophins, respectively. Neither fragment was amplified from the lymphoblastoid cells of a patient with Duchenne muscular dystrophy, who has a partial deletion involving the brain- and muscle-type promoters in the dystrophin gene. These findings showed that the brain-type as well as the muscle-type promoter of the dystrophin gene was active in lymphocytes and lymphoblastoid cells.

Publication types

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

MeSH terms

  • B-Lymphocytes / metabolism
  • B-Lymphocytes / microbiology
  • Base Sequence
  • Brain / metabolism*
  • Cell Line, Transformed
  • Cell Transformation, Viral
  • DNA, Complementary / genetics
  • Dystrophin / genetics*
  • Exons
  • Gene Expression Regulation
  • Genes
  • Herpesvirus 4, Human
  • Humans
  • Intellectual Disability / genetics*
  • Lymphocytes / metabolism*
  • Male
  • Molecular Sequence Data
  • Muscle Proteins / genetics*
  • Muscles / metabolism*
  • Muscular Dystrophies / genetics*
  • Muscular Dystrophies / pathology
  • Nerve Tissue Proteins / genetics*
  • Organ Specificity
  • Polymerase Chain Reaction
  • Promoter Regions, Genetic*
  • Sequence Deletion
  • Sequence Homology, Nucleic Acid

Substances

  • DNA, Complementary
  • Dystrophin
  • Muscle Proteins
  • Nerve Tissue Proteins