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Titolo:
Neurodegenerative tauopathies
Autore:
Lee, VMY; Goedert, M; Trojanowski, JQ;
Indirizzi:
Univ Penn, Sch Med, Dept Pathol & Lab Med, Ctr Neurodegenerat Dis Res, Philadelphia, PA 19104 USA Univ Penn Philadelphia PA USA 19104 t Dis Res, Philadelphia, PA 19104 USA MRC, Mol Biol Lab, Cambridge CB2 2QH, England MRC Cambridge England CB2 2QH , Mol Biol Lab, Cambridge CB2 2QH, England
Titolo Testata:
ANNUAL REVIEW OF NEUROSCIENCE
, volume: 24, anno: 2001,
pagine: 1121 - 1159
SICI:
0147-006X(2001)24:<1121:NT>2.0.ZU;2-X
Fonte:
ISI
Lingua:
ENG
Soggetto:
PROGRESSIVE SUPRANUCLEAR PALSY; MICROTUBULE-ASSOCIATED PROTEIN; PAIRED HELICAL FILAMENTS; GLYCOGEN-SYNTHASE KINASE-3-BETA; CYCLIN-DEPENDENT KINASE-5; FAMILIAL FRONTOTEMPORAL DEMENTIA; RICHARDSON-OLSZEWSKI-SYNDROME; PERIPHERAL NERVOUS-SYSTEM; ALZHEIMER-LIKE PHOSPHORYLATION; REDUCES TAU-PHOSPHORYLATION;
Keywords:
Alzheimer's disease; frontotemporal dementia; mutation; neurodegenerative disease; filamentous deposits; pathology; tau protein;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
256
Recensione:
Indirizzi per estratti:
Indirizzo: Lee, VMY Univ Penn, Sch Med, Dept Pathol & Lab Med, Ctr Neurodegenerat DisRes, Philadelphia, PA 19104 USA Univ Penn Philadelphia PA USA 19104 , Philadelphia, PA 19104 USA
Citazione:
V.M.Y. Lee et al., "Neurodegenerative tauopathies", ANN R NEUR, 24, 2001, pp. 1121-1159

Abstract

The defining neuropathological characteristics of Alzheimer's disease are abundant filamentous tau lesions and deposits of fibrillar amyloid beta peptides. Prominent filamentous tau inclusions and brain degeneration in the absence of beta -amyloid deposits are also hallmarks of neurodegenerative tauopathies exemplified by sporadic corticobasal degeneration, progressive supranuclear palsy, and Pick's disease, as well as by hereditary frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). Because multiple tau gene mutations are pathogenic for FTDP-17 and tau polymorphisms appear to be genetic risk factors for sporadic progressive supranuclear palsy and corticobasal degeneration, tau abnormalities are linked directly to the etiology and pathogenesis of neurodegenerative disease. Indeed, emergingdata support the hypothesis that different tau gene mutations are pathogenic because they impair tau functions, promote tau fibrillization, or perturb tau gene splicing, thereby leading to formation of biochemically and structurally distinct aggregates of tau. Nonetheless, different members of the same kindred often exhibit diverse FTDP-17 syndromes, which suggests that additional genetic or epigenetic factors influence the phenotypic manifestations of neurodegenerative tauopathies. Although these and other hypothetical mechanisms of neurodegenerative tauopathies remain to be tested and validated, transgenic models are increasingly available for this purpose, and they will accelerate discovery of more effective therapies for neurodegenerative tauopathies and related disorders, including Alzheimer's disease.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 26/01/20 alle ore 22:30:50