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Titolo:
Mice containing a human chromosome 21 model behavioral impairment and cardiac anomalies of Down's syndrome
Autore:
Shinohara, T; Tomizuka, K; Miyabara, S; Takehara, S; Kazuki, Y; Inoue, J; Katoh, M; Nakane, H; Iino, A; Ohguma, A; Ikegami, S; Inokuchi, K; Ishida, I; Reeves, RH; Oshimura, M;
Indirizzi:
Tottori Univ, Fac Med, Dept Mol & Cell Genet, Sch Life Sci, Yonago, Tottori 6838503, Japan Tottori Univ Yonago Tottori Japan 6838503 Yonago, Tottori 6838503, Japan CREST, JST, Yonago, Tottori 6838503, Japan CREST Yonago Tottori Japan 6838503 T, JST, Yonago, Tottori 6838503, Japan Kirin Brewery Co Ltd, Pharmaceut Res Lab, Takasaki, Gumma 3701295, Japan Kirin Brewery Co Ltd Takasaki Gumma Japan 3701295 i, Gumma 3701295, Japan Saga Med Sch, Dept Pathol, Saga 8498501, Japan Saga Med Sch Saga Japan 8498501 ed Sch, Dept Pathol, Saga 8498501, Japan Tottori Univ, Fac Med, Dept Anat 1, Yonago, Tottori 6838503, Japan TottoriUniv Yonago Tottori Japan 6838503 Yonago, Tottori 6838503, Japan Mitsubishi Kasei Inst Life Sci, Tokyo 1948511, Japan Mitsubishi Kasei InstLife Sci Tokyo Japan 1948511 Tokyo 1948511, Japan Johns Hopkins Univ, Sch Med, Dept Physiol, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 hysiol, Baltimore, MD 21205 USA
Titolo Testata:
HUMAN MOLECULAR GENETICS
fascicolo: 11, volume: 10, anno: 2001,
pagine: 1163 - 1175
SICI:
0964-6906(20010515)10:11<1163:MCAHC2>2.0.ZU;2-2
Fonte:
ISI
Lingua:
ENG
Soggetto:
CHIMERIC MICE; CELL-LINES; MOUSE; GENE; HUMAN-CHROMOSOME-21; REGION; EXPRESSION; FRAGMENTS; DEFECTS; RATS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
49
Recensione:
Indirizzi per estratti:
Indirizzo: Oshimura, M Tottori Univ, Fac Med, Dept Mol & Cell Genet, Sch Life Sci, Nishimachi 86,Yonago, Tottori 6838503, Japan Tottori Univ Nishimachi 86 Yonago Tottori Japan 6838503 Japan
Citazione:
T. Shinohara et al., "Mice containing a human chromosome 21 model behavioral impairment and cardiac anomalies of Down's syndrome", HUM MOL GEN, 10(11), 2001, pp. 1163-1175

Abstract

Trisomy 21 (Ts21) is the most common live-born human aneuploidy; it results in a constellation of features known as Down's syndrome (DS), Ts21 is themost frequent cause of congenital heart defects and the leading genetic cause of mental retardation. To investigate the gene dosage effects of an extra copy of human chromosome 21 (Chr 21) on various phenotypes, we used microcell-mediated chromosome transfer to create embryonic stem (ES) cells containing Chr 21, ES cell lines retaining Chr 21 as an independent chromosome were used to produce chimeric mice with a substantial contribution from Chr21-containing cells. Fluorescence in situ hybridization and PCR-based DNA analysis revealed that Chr 21 was substationally intact but had sustained asmall deletion. The freely segregating Chr 21 was lost during development in some tissues, resulting in a panel of chimeric mice with various mosaicism as regards retention of the Chr 21, These chimeric mice showed a high correlation between retention of Chr 21 in the brain and impairment in learning or emotional behavior by open-field, contextual fear conditioning and forced swim tests. Hypoplastic thymus and cardiac defects, i.e. double outletright ventricle and riding aorta, were observed in a considerable number of chimeric mouse fetuses with a high contribution of Chr 21. These chimericmice mimic a wide variety of phenotypic traits of DS, revealing the utility of mice containing Chr 21 as unique models for DS and for the identification of genes responsible for DS.

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Documento generato il 01/04/20 alle ore 00:45:44