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Titolo:
Progressive cerebellar, auditory, and esophageal dysfunction caused by targeted disruption of the frizzled-4 gene
Autore:
Wang, YS; Huso, D; Cahill, H; Ryugo, D; Nathans, J;
Indirizzi:
Johns Hopkins Univ, Sch Med, Dept Mol Biol & Genet, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 Genet, Baltimore, MD 21205 USA Johns Hopkins Univ, Sch Med, Div Comparat Med, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 at Med, Baltimore, MD 21205 USA Johns Hopkins Univ, Sch Med, Dept Otolaryngol Head & Neck Surg, Baltimore,MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 ck Surg, Baltimore,MD 21205 USA Johns Hopkins Univ, Sch Med, Dept Neurosci, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 urosci, Baltimore, MD 21205 USA Johns Hopkins Univ, Sch Med, Dept Ophthalmol, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 halmol, Baltimore, MD 21205 USA Johns Hopkins Univ, Sch Med, Howard Hughes Med Inst, Baltimore, MD 21205 USA Johns Hopkins Univ Baltimore MD USA 21205 d Inst, Baltimore, MD 21205 USA
Titolo Testata:
JOURNAL OF NEUROSCIENCE
fascicolo: 13, volume: 21, anno: 2001,
pagine: 4761 - 4771
SICI:
0270-6474(20010701)21:13<4761:PCAAED>2.0.ZU;2-O
Fonte:
ISI
Lingua:
ENG
Soggetto:
GRANULE CELL-DEATH; BETA-CATENIN; INT-1 PROTOONCOGENE; NERVOUS-SYSTEM; STAGGERER GENE; MUTANT MOUSE; WNT GENES; EXPRESSION; MICE; WINGLESS;
Keywords:
frizzled-4; cerebellar degeneration; Purkinje cells; Wnt signaling; esophagus; progressive hearing loss;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
64
Recensione:
Indirizzi per estratti:
Indirizzo: Nathans, J Johns Hopkins Univ, Sch Med, Dept Mol Biol & Genet, 805 PCTB,725 N Wolfe St, Baltimore, MD 21205 USA Johns Hopkins Univ 805 PCTB,725 N Wolfe St Baltimore MD USA 21205
Citazione:
Y.S. Wang et al., "Progressive cerebellar, auditory, and esophageal dysfunction caused by targeted disruption of the frizzled-4 gene", J NEUROSC, 21(13), 2001, pp. 4761-4771

Abstract

Wnt signaling has been implicated in the control of cell proliferation andin synapse formation during neural development, and these actions are presumed to be mediated by frizzled receptors. In this paper we report the phenotype of mice carrying a targeted deletion of the frizzled-4 (fz4) gene. fz4(-/-) mice exhibit three distinct defects: (1) progressive cerebellar degeneration associated with severe ataxia, (2) absence of a skeletal muscle sheath around the lower esophagus associated with progressive esophageal distension and dysfunction, and (3) progressive deafness caused by a defect in the peripheral auditory system unaccompanied by loss of hair cells or otherauditory neurons. As assayed using a lacZ knock-in reporter, fz4 is widelyexpressed within the CNS. In particular, fz4 is expressed in cerebellar Purkinje cells, esophageal skeletal muscle, and cochlear inner hair cells, and the absence of Fz4 in these cells is presumed to account for the fz4(-/-)phenotype. In contrast to the early cell proliferation and patterning effects classically ascribed to Wnts, the auditory and cerebellar phenotypes offz4(-/-) mice implicate Frizzled signaling in maintaining the viability and integrity of the nervous system in later life.

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Documento generato il 04/12/20 alle ore 09:52:57