PT  - JOURNAL ARTICLE
AU  - Gussoni, E
AU  - Pavlath, G K
AU  - Miller, R G
AU  - Panzara, M A
AU  - Powell, M
AU  - Blau, H M
AU  - Steinman, L
TI  - Specific T cell receptor gene rearrangements at the site of muscle degeneration in Duchenne muscular dystrophy.
DP  - 1994 Nov 15
TA  - The Journal of Immunology
PG  - 4798--4805
VI  - 153
IP  - 10
4099  - http://www.jimmunol.org/content/153/10/4798.short
4100  - http://www.jimmunol.org/content/153/10/4798.full
SO  - J. Immunol.1994 Nov 15; 153
AB  - 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.