SPECIFIC T-CELL RECEPTOR GENE REARRANGEMENTS AT THE SITE OF MUSCLE DEGENERATION IN DUCHENNE MUSCULAR-DYSTROPHY

Citation
E. Gussoni et al., SPECIFIC T-CELL RECEPTOR GENE REARRANGEMENTS AT THE SITE OF MUSCLE DEGENERATION IN DUCHENNE MUSCULAR-DYSTROPHY, The Journal of immunology, 153(10), 1994, pp. 4798-4805
Citations number
37
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Immunology
Journal title
The Journal of immunology
ISSN journal
0022-1767 → ACNP
Volume
153
Issue
10
Year of publication
1994
Pages
4798 - 4805
Database
ISI
SICI code
0022-1767(1994)153:10<4798:STRGRA>2.0.ZU;2-E
Abstract
Mononuclear cells infiltrate degenerating muscles of Duchenne muscular dystrophy (DMD) patients. Using a quantitative PCR, we first characte rized the T cells infiltrating muscle biopsies from six DMD patients. High levels of TCR V beta 2 transcripts were observed in DMD muscle ti ssue. TCR V beta 2 transcripts from seven DMD patients and five contro ls were sequenced, and the VDJ junctional region analyzed in 166 clone s. One specific amino acid motif, RVSG, was found in the third complem entary determining region (CDR3) of TCR V beta 2 chains in samples fro m five DMD patients, but not in controls. A specific immune reaction a t the site of tissue degeneration may play an important role in the pa thogenesis of DMD.