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Titolo:
ABNORMAL ACCUMULATION OF PRION PROTEIN MESSENGER-RNA IN MUSCLE-FIBERSOF PATIENTS WITH SPORADIC INCLUSION-BODY MYOSITIS AND HEREDITARY INCLUSION-BODY MYOPATHY
Autore:
SARKOZI E; ASKANAS V; ENGEL WK;
Indirizzi:
UNIV SO CALIF,SCH MED,CTR NEUROMUSCULAR,DEPT NEUROL,637 S LUCAS AVE LOS ANGELES CA 90017 UNIV SO CALIF,SCH MED,CTR NEUROMUSCULAR,DEPT NEUROL LOS ANGELES CA 90017
Titolo Testata:
The American journal of pathology
fascicolo: 6, volume: 145, anno: 1994,
pagine: 1280 - 1284
SICI:
0002-9440(1994)145:6<1280:AAOPPM>2.0.ZU;2-Y
Fonte:
ISI
Lingua:
ENG
Soggetto:
SCRAPIE AGENT PROTEIN; BETA-AMYLOID PROTEIN; MESSENGER-RNA; PRECURSOR; DISEASES; BIOPSIES; GENE;
Tipo documento:
Note
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
27
Recensione:
Indirizzi per estratti:
Citazione:
E. Sarkozi et al., "ABNORMAL ACCUMULATION OF PRION PROTEIN MESSENGER-RNA IN MUSCLE-FIBERSOF PATIENTS WITH SPORADIC INCLUSION-BODY MYOSITIS AND HEREDITARY INCLUSION-BODY MYOPATHY", The American journal of pathology, 145(6), 1994, pp. 1280-1284

Abstract

Sporadic inclusion-body myositis is the most common Progressive muscle disease of older patients. The muscle biopsy demonstrates mononuclear cell inflammation and vacuolated muscle fibers containing paired helical filaments and 6 to 10-nm fibrils, both resembling those of Alzheimer brain, and Congo-red positivity. Hereditary inclusion-body myopathy designates patients cytopathologically similar but without inflammation. In both muscle diseases, prion, and several proteins characteristic of Alzheimer brain-eg, beta amyloid protein and hyperphosphorylatedtau (which normally are expressed mainly in neurons), and apolipoprotein E-are abnormally accumulated in vacuolated muscle fibers, by unknown mechanisms. We now demonstrate in both muscle diseases that prion mRNA is strongly expressed ill the vacuolated muscle fibers, which suggests that their accumulated prion protein results, at least partly, from increased gene expression This, to our knowledge, is the first demonstration of abnormally increased prion mRNA in human disease. Anothernovel finding is the increased prion mRNA in human muscle macrophages, and both increased prion protein and prion mRNA ill regenerating muscle fibers. The latter indicates that prion may play a role in human muscle development.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 04/12/20 alle ore 02:37:05