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Titolo:
Prion diseases: What is the neurotoxic molecule?
Autore:
Chiesa, R; Harris, DA;
Indirizzi:
Ist Ric Farmacol Mario Negri, I-20157 Milan, Italy Ist Ric Farmacol Mario Negri Milan Italy I-20157 i, I-20157 Milan, Italy Washington Univ, Sch Med, Dept Cell Biol & Physiol, St Louis, MO 63110 USAWashington Univ St Louis MO USA 63110 l & Physiol, St Louis, MO 63110 USA
Titolo Testata:
NEUROBIOLOGY OF DISEASE
fascicolo: 5, volume: 8, anno: 2001,
pagine: 743 - 763
SICI:
0969-9961(200110)8:5<743:PDWITN>2.0.ZU;2-Q
Fonte:
ISI
Lingua:
ENG
Soggetto:
CREUTZFELDT-JAKOB-DISEASE; CEREBELLAR GRANULE CELLS; FATAL FAMILIAL INSOMNIA; CENTRAL-NERVOUS-SYSTEM; DISRUPTED PRP GENE; PROTEIN NULL MICE; TRANSGENIC MICE; ENDOPLASMIC-RETICULUM; CULTURED-CELLS; SPONGIFORM ENCEPHALOPATHY;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
174
Recensione:
Indirizzi per estratti:
Indirizzo: Chiesa, R Ist Ric Farmacol Mario Negri, Via Eritrea 62, I-20157 Milan, Italy Ist Ric Farmacol Mario Negri Via Eritrea 62 Milan Italy I-20157
Citazione:
R. Chiesa e D.A. Harris, "Prion diseases: What is the neurotoxic molecule?", NEUROBIOL D, 8(5), 2001, pp. 743-763

Abstract

A great deal of effort has been devoted during the past 20 years to defining the chemical nature of prions, the infectious agents responsible for transmissible spongiform encephalopathies. In contrast, much less attention has been paid to elucidating how prions actually damage the central nervous system. Although it is commonly assumed that PrPSc, the protein constituent of infectious prions, is the primary culprit, increasing evidence indicatesthat this may not be the case. Several alternative molecular forms of PrP are reasonable candidates for the neurotoxic species in prion diseases, although it is still too early to tell whether these or other ones will turn out to be the true instigating factors. The cellular pathways activated by neurotoxic forms of PrP that ultimately result in neuronal death are also being investigated, and several possible mechanisms have been uncovered, including the operation of quality control processes in the endoplasmic reticulum. Elucidating the distinction between the infectious and neurotoxic formsof PrP has important implications for designing therapy of prion diseases,as well as for understanding pathogenic mechanisms operative in other neurodegenerative disorders and the role of prion-like states in biology. (C) 2001 Academic Press.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/04/20 alle ore 02:21:59