Specific T cell receptor gene rearrangements at the site of muscle degeneration in Duchenne muscular dystrophy.
E Gussoni, GK Pavlath, RG Miller… - … (Baltimore, Md.: 1950 …, 1994 - journals.aai.org
Journal of immunology (Baltimore, Md.: 1950), 1994•journals.aai.org
Mononuclear cells infiltrate degenerating muscles of Duchenne muscular dystrophy (DMD)
patients. Using a quantitative PCR, we first characterized the T cells infiltrating muscle
biopsies from six DMD patients. High levels of TCR V beta 2 transcripts were observed in
DMD muscle tissue. TCR V beta 2 transcripts from seven DMD patients and five controls
were sequenced, and the VDJ junctional region analyzed in 166 clones. One specific amino
acid motif, RVSG, was found in the third complementary determining region (CDR3) of TCR …
patients. Using a quantitative PCR, we first characterized the T cells infiltrating muscle
biopsies from six DMD patients. High levels of TCR V beta 2 transcripts were observed in
DMD muscle tissue. TCR V beta 2 transcripts from seven DMD patients and five controls
were sequenced, and the VDJ junctional region analyzed in 166 clones. One specific amino
acid motif, RVSG, was found in the third complementary determining region (CDR3) of TCR …
Abstract
Mononuclear cells infiltrate degenerating muscles of Duchenne muscular dystrophy (DMD) patients. Using a quantitative PCR, we first characterized the T cells infiltrating muscle biopsies from six DMD patients. High levels of TCR V beta 2 transcripts were observed in DMD muscle tissue. TCR V beta 2 transcripts from seven DMD patients and five controls were sequenced, and the VDJ junctional region analyzed in 166 clones. One specific amino acid motif, RVSG, was found in the third complementary determining region (CDR3) of TCR V beta 2 chains in samples from five DMD patients, but not in controls. A specific immune reaction at the site of tissue degeneration may play an important role in the pathogenesis of DMD.
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