PMID- 9865793
OWN - NLM
STAT- MEDLINE
DCOM- 19990107
LR  - 20190717
IS  - 0003-9942 (Print)
IS  - 0003-9942 (Linking)
VI  - 55
IP  - 12
DP  - 1998 Dec
TI  - Molecular immunology and genetics of inflammatory muscle diseases.
PG  - 1509-12
AB  - Polymyositis, dermatomyositis, and inclusion body myositis, although 
      immunopathologically distinct, share 3 dominant histological features: 
      inflammation, fibrosis, and loss of muscle fibers. Progress in molecular 
      immunology and immunogenetics has enhanced our understanding of these cellular 
      processes. Based on the T-cell receptor gene rearrangement, the autoinvasive CD8+ 
      T cells in polymyositis and inclusion body myositis, but not dermatomyositis, are 
      specifically selected and clonally expanded in situ by heretofore unknown 
      muscle-specific autoantigens. The messenger RNA of cytokines is variably 
      expressed, except for a persistent up-regulation of interleukin 1beta in 
      inclusion body myositis and transforming growth factor beta in dermatomyositis. 
      In inclusion body myositis, the interleukin 1, secreted by the chronically 
      activated endomysial inflammatory cells, may participate in the formation of 
      amyloid because it up-regulates beta-amyloid precursor protein (beta-APP) gene 
      expression and beta-APP promoter and colocalizes with beta-APP within the 
      vacuolated muscle fibers. In dermatomyositis, transforming growth factor beta is 
      overexpressed in the perimysial connective tissue but is down-regulated after 
      successful immunotherapy and reduction of inflammation and fibrosis. The 
      degenerating muscle fibers express several antiapoptotic molecules, such as 
      Bcl-2, and resist apoptosis-mediated cell death. In myositis, several of the 
      identified molecules and adhesion receptors play a role in the process of 
      inflammation, fibrosis, and muscle fiber loss, and could be targets for the 
      design of semispecific therapeutic interventions.
FAU - Dalakas, M C
AU  - Dalakas MC
AD  - Neuromuscular Diseases Section, National Institute of Neurological Disorders and 
      Stroke, National Institutes of Health, Bethesda, MD 20892-1382, USA.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Arch Neurol
JT  - Archives of neurology
JID - 0372436
RN  - 0 (Autoantigens)
RN  - 0 (Cell Adhesion Molecules)
RN  - 0 (Cytokines)
RN  - 0 (RNA, Messenger)
SB  - IM
MH  - Apoptosis
MH  - Autoantigens/*genetics/immunology
MH  - Cell Adhesion Molecules/immunology/pharmacology
MH  - Cytokines/biosynthesis/genetics/*immunology
MH  - Dermatomyositis/*genetics/*immunology
MH  - Down-Regulation
MH  - Fibrosis/immunology/physiopathology
MH  - *Gene Expression Regulation
MH  - Gene Rearrangement, T-Lymphocyte
MH  - Humans
MH  - Inclusion Bodies
MH  - Inflammation/immunology
MH  - Muscle Fibers, Skeletal/immunology/pathology
MH  - Polymyositis/*genetics/*immunology
MH  - RNA, Messenger/biosynthesis/genetics
RF  - 22
EDAT- 1998/12/29 00:00
MHDA- 1998/12/29 00:01
CRDT- 1998/12/29 00:00
PHST- 1998/12/29 00:00 [pubmed]
PHST- 1998/12/29 00:01 [medline]
PHST- 1998/12/29 00:00 [entrez]
AID - 10.1001/archneur.55.12.1509 [doi]
PST - ppublish
SO  - Arch Neurol. 1998 Dec;55(12):1509-12. doi: 10.1001/archneur.55.12.1509.