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Titolo:
Mutation frequency is reduced in the cerebellum of Big Blue (R) mice overexpressing a human wild type SOD1 gene
Autore:
Kunishige, M; Hill, KA; Riemer, AM; Farwell, KD; Halangoda, A; Heinmoller, E; Moore, SR; Turner, DM; Sommer, SS;
Indirizzi:
City Hope Beckman Res Inst, Dept Mol Genet, Duarte, CA 91010 USA City HopeBeckman Res Inst Duarte CA USA 91010 enet, Duarte, CA 91010 USA
Titolo Testata:
MUTATION RESEARCH-FUNDAMENTAL AND MOLECULAR MECHANISMS OF MUTAGENESIS
fascicolo: 2, volume: 473, anno: 2001,
pagine: 139 - 149
SICI:
1386-1964(20010220)473:2<139:MFIRIT>2.0.ZU;2-N
Fonte:
ISI
Lingua:
ENG
Soggetto:
AMYOTROPHIC-LATERAL-SCLEROSIS; CUZN-SUPEROXIDE-DISMUTASE; MOTOR-NEURON DISEASE; TRANSGENIC MOUSE MODELS; FOCAL CEREBRAL-ISCHEMIA; OXIDATIVE DAMAGE; DETECTION SYSTEM; SHUTTLE VECTOR; ANIMAL-MODEL; INJURY;
Keywords:
Lac1; superoxide dismutase; amyotrophic lateral sclerosis; oxidative damage; neurodegeneration;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
55
Recensione:
Indirizzi per estratti:
Indirizzo: Sommer, SS City Hope Beckman Res Inst, Dept Mol Genet, Duarte, CA 91010 USA City Hope Beckman Res Inst Duarte CA USA 91010 e, CA 91010 USA
Citazione:
M. Kunishige et al., "Mutation frequency is reduced in the cerebellum of Big Blue (R) mice overexpressing a human wild type SOD1 gene", MUT RES-F M, 473(2), 2001, pp. 139-149

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive paralytic disorder caused by motor neuron degeneration. A similar disease phenotype is observed in mice overexpressing a mutant human hSOD1 gene(G93A, 1Gurd(1)). Mice transgenic for lad (Big Blue(R)) and human mutant (1Gurd(1), Mut hSOD1) or wildtype (2Gur, Wt hSOD1) SOD1 genes were used to examine spontaneous mutation, oxidative DNA damage. and neurodegeneration in vivo. The frequency and pattern of spontaneous mutation were determined for forebrain (90% glia), cerebellum (90% neurons) and thymus from 5-month-old male mice. Mutation frequency is not elevated significantly and mutation pattern is unaltered in MuthSOD1 mice compared to control mice. Mutation frequency is reduced significantly in the cerebellum of Wt hSOD1 mice (1.6 x 10(-5). P = 0.0093; Fisher's Exact Test) compared to mice without a human transgene (2.7 x 10(-5)). Mutation pattern is unaltered. This first report of an endogenous factor that can reduce in vivo, the frequency of spontaneous mutation suggests potential strategies for lowering mutagenesis related to aging, neurodegeneration, and carcinogenesis. (C) 2001 Elsevier Science B.V. All rights reserved.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 23/10/20 alle ore 14:40:13