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Titolo:
The ubiquitin protein catabolic disorders
Autore:
Layfield, R; Alban, A; Mayer, RJ; Lowe, J;
Indirizzi:
Univ Nottingham, Sch Med, Queens Med Ctr, Sch Biomed Sci, Nottingham NG7 2UH, England Univ Nottingham Nottingham England NG7 2UH , Nottingham NG7 2UH, England Univ Nottingham, Sch Med, Queens Med Ctr, Sch Clin Lab Sci, Nottingham NG72UH, England Univ Nottingham Nottingham England NG7 2UH i, Nottingham NG72UH, England
Titolo Testata:
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
fascicolo: 3, volume: 27, anno: 2001,
pagine: 171 - 179
SICI:
0305-1846(200106)27:3<171:TUPCD>2.0.ZU;2-1
Fonte:
ISI
Lingua:
ENG
Soggetto:
RECESSIVE JUVENILE PARKINSONISM; CARBOXY-TERMINAL HYDROLASE; TUMOR-SUPPRESSOR PROTEIN; ALPHA-SYNUCLEIN; ALZHEIMERS-DISEASE; INCLUSION-BODIES; NEURODEGENERATIVE DISEASES; PROTEASOMAL DEGRADATION; INTRANUCLEAR INCLUSIONS; CONJUGATING ENZYME;
Keywords:
ubiquitin; proteasome; neurodegeneration; Alzheimer's disease;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
54
Recensione:
Indirizzi per estratti:
Indirizzo: Layfield, R Univ Nottingham, Sch Med, Queens Med Ctr, Sch Biomed Sci, D Floor, Nottingham NG7 2UH, England Univ Nottingham D Floor Nottingham England NG7 2UH H, England
Citazione:
R. Layfield et al., "The ubiquitin protein catabolic disorders", NEUROP AP N, 27(3), 2001, pp. 171-179

Abstract

The ubiquitin-proteasome system of intracellular proteolysis is essential for cell viability. We propose the concept that neurodegenerative diseases such as Alzheimer's and Parkinson's, as well as other conditions including some types of cancer, collectively represent a raft of 'ubiquitin protein catabolic disorders' in which altered function of the ubiquitin-proteasome system can cause or directly contribute to disease pathogenesis. Genetic abnormalities within the ubiquitin pathway, either in ubiquitin-ligase (E3) enzymes or in deubiquitinating enzymes, cause disease because of problems associated with substrate recognition or supply of free ubiquitin, respectively. In some cases, mutations in protein substrates of the ubiquitin-proteasome system may directly contribute to disease progression because of inefficient substrate recognition. Mutations in transcripts for the ubiquitin protein itself (as a result of 'molecular misreading') also affect ubiquitin-dependent proteolysis with catastrophic consequences. This has been shown in Alzheimer's disease and could apply to other age-associated neurodegenerative conditions. Within the nervous system, accumulation of unwanted proteinsas a result of defective ubiquitin-dependent proteolysis may contribute toaggregation events, which underlie the pathogenesis of several major humanneurodegenerative diseases.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 17/09/19 alle ore 18:30:08