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Titolo:
Loss of cortical and thalamic neuronal tenascin-C expression in a transgenic mouse expressing exon 1 of the human Huntington disease gene
Autore:
Kusakabe, M; Mangiarini, L; Laywell, ED; Bates, GP; Yoshiki, A; Hiraiwa, N; Inoue, J; Steindler, DA;
Indirizzi:
Univ Tennessee, Coll Med, Dept Neurobiol & Anat, Memphis, TN 38163 USA Univ Tennessee Memphis TN USA 38163 urobiol & Anat, Memphis, TN 38163 USA RIKEN, Div Expt Anim Res, Tsukuba, Ibaraki, Japan RIKEN Tsukuba Ibaraki Japan , Div Expt Anim Res, Tsukuba, Ibaraki, Japan Univ London Kings Coll, Div Med & Mol Genet, London WC2R 2LS, England UnivLondon Kings Coll London England WC2R 2LS London WC2R 2LS, England
Titolo Testata:
JOURNAL OF COMPARATIVE NEUROLOGY
fascicolo: 4, volume: 430, anno: 2001,
pagine: 485 - 500
SICI:
0021-9967(20010219)430:4<485:LOCATN>2.0.ZU;2-Q
Fonte:
ISI
Lingua:
ENG
Soggetto:
N-TERMINAL HUNTINGTIN; EXPANDED CAG REPEAT; SUBVENTRICULAR ZONE; OLFACTORY-BULB; NERVOUS-SYSTEM; INTRANUCLEAR INCLUSIONS; GLUTAMINE REPEATS; SUBEPENDYMAL ZONE; CEREBRAL-CORTEX; ADULT-MOUSE;
Keywords:
Huntington's disease; tenascin-C; transgenic mice; thalamostriatal and corticostriatal neurons; in situ hybridization; immunocytochemistry;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
67
Recensione:
Indirizzi per estratti:
Indirizzo: Steindler, DA Univ Tennessee, Coll Med, Dept Neurobiol & Anat, 855 Monroe Ave, Memphis, TN 38163 USA Univ Tennessee 855 Monroe Ave Memphis TN USA 38163 38163 USA
Citazione:
M. Kusakabe et al., "Loss of cortical and thalamic neuronal tenascin-C expression in a transgenic mouse expressing exon 1 of the human Huntington disease gene", J COMP NEUR, 430(4), 2001, pp. 485-500

Abstract

A transgenic mouse containing the first exon of the human Huntington's disease (HD) gene has revealed a variety of behavioral and pathophysiological anomalies reminiscent of certain aspects of human Huntington's disease (HD). The present study has found that expression of the extracellular matrix glycoprotein tenascin-C appears to be unaffected in astroglial cells in wild-type and R6/2 transgenic mice that express the mutant huntingtin protein but that it is conspicuously absent in two neuronal populations within the cerebral cortex and thalamus of the R6/2 mice. Loss of tenascin-C expressionbegins between the fourth and eighth postnatal weeks, coincidental with the onset of abnormal behavioral phenotype and the appearance of intranuclearinclusion bodies and neuropil aggregates. By 12 weeks, R6/2 mice exhibit acomplete absence of tenascin-C neuronal immunolabeling, a disappearance ofcRNA probe-positive neurons across discrete cytoarchitectonic regions of the dorsal thalamus (e.g., the ventromedial, parafascicular, lateral posterior, and posterior thalamic groups) and frontal cortex, and an accompanying thalamic astrogliosis. The loss of neuronal tenascin-C expression includes structures that are known to send converging excitatory axonal projections to the caudate-putamen, the structure that is most at risk for neurodegeneration in HD. Altered neuronal expression of tenascin-C in R6/2 mice implicates altered transcriptional activities of the mutant huntingtin protein. The abnormal biochemistry and possibly abnormal activity of thalamostriate and corticostriate projection neurons may also affect abnormal neuronal activities in their primary connectional target, the neostriatum, which is severely compromised in HD. (C) 2001 Wiley-Liss, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 07/07/20 alle ore 18:19:09