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Titolo:
Mutant and wild type human alpha-synucleins assemble into elongated filaments with distinct morphologies in vitro
Autore:
Giasson, BI; Uryu, K; Trojanowski, JQ; Lee, VMY;
Indirizzi:
Univ Penn, Sch Med, Dept Pathol & Lab Med, Philadelphia, PA 19104 USA UnivPenn Philadelphia PA USA 19104 & Lab Med, Philadelphia, PA 19104 USA
Titolo Testata:
JOURNAL OF BIOLOGICAL CHEMISTRY
fascicolo: 12, volume: 274, anno: 1999,
pagine: 7619 - 7622
SICI:
0021-9258(19990319)274:12<7619:MAWTHA>2.0.ZU;2-U
Fonte:
ISI
Lingua:
ENG
Soggetto:
MULTIPLE SYSTEM ATROPHY; OLIGODENDROGLIAL CYTOPLASMIC INCLUSIONS; PARKINSONS-DISEASE; LEWY BODIES; OLIVOPONTOCEREBELLAR ATROPHY; ALZHEIMERS-DISEASE; NERVOUS-SYSTEM; PROTEIN; COMPONENT; EPITOPES;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
43
Recensione:
Indirizzi per estratti:
Indirizzo: Lee, VMY Hosp3,niv Penn, Dept Pathol & Lab Med, Ctr Neurodegenerat Dis Res, Maloney Hosp Univ Penn Maloney 3 Philadelphia PA USA 19104 s Res, Maloney
Citazione:
B.I. Giasson et al., "Mutant and wild type human alpha-synucleins assemble into elongated filaments with distinct morphologies in vitro", J BIOL CHEM, 274(12), 1999, pp. 7619-7622

Abstract

alpha-Synuclein is a soluble presynaptic protein which is pathologically redistributed within intracellular lesions characteristic of several neurodegenerative diseases. Here we demonstrate that wild type and two mutant forms of alpha-synuclein linked to familial Parkinson's disease (Ala(30) --> Pro and Ala(53) --> Thr) self-aggregate and assemble into 10-19-nm-wide filaments with distinct morphologies under defined in vitro conditions. Immunogold labeling demonstrates that the central region of all these filaments aremore robustly labeled than the N-terminal or C-terminal regions, suggesting that the latter regions are buried within the filaments. Since in vitro generated alpha-synuclein filaments resemble the major ultrastructural elements of authentic Lewy bodies that are hallmark lesions of Parkinson's disease, we propose that self-aggregating alpha-synuclein is the major subunit protein of these filamentous lesions.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 07/07/20 alle ore 15:36:40