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Titolo:
Rnx deficiency results in congenital central hypoventilation
Autore:
Shirasawa, S; Arata, A; Onimaru, H; Roth, KA; Brown, GA; Horning, S; Arata, S; Okumura, K; Sasazuki, T; Korsmeyer, SJ;
Indirizzi:
Washington Univ, Sch Med, Dept Med, Howard Hughes Med Inst, St Louis, MO 63110 USA Washington Univ St Louis MO USA 63110 es Med Inst, St Louis, MO 63110 USA Washington Univ, Sch Med, Dept Pathol, Howard Hughes Med Inst, St Louis, MO 63110 USA Washington Univ St Louis MO USA 63110 es Med Inst, St Louis, MO 63110 USA Kyushu Univ, Med Inst Bioregulat, Dept Genet, Fukuoka 812, Japan Kyushu Univ Fukuoka Japan 812 Bioregulat, Dept Genet, Fukuoka 812, Japan Tokyo Metropolitan Inst Neurosci, Dept Neurobiol, Tokyo 138, Japan Tokyo Metropolitan Inst Neurosci Tokyo Japan 138 obiol, Tokyo 138, Japan Showa Univ, Sch Med, Dept Physiol, Tokyo 142, Japan Showa Univ Tokyo Japan 142 Univ, Sch Med, Dept Physiol, Tokyo 142, Japan Showa Univ, Ctr Biotechnol, Tokyo 142, Japan Showa Univ Tokyo Japan 142Showa Univ, Ctr Biotechnol, Tokyo 142, Japan Harvard Univ, Sch Med, Dana Farber Canc Inst, Boston, MA 02115 USA HarvardUniv Boston MA USA 02115 a Farber Canc Inst, Boston, MA 02115 USA
Titolo Testata:
NATURE GENETICS
fascicolo: 3, volume: 24, anno: 2000,
pagine: 287 - 290
SICI:
1061-4036(200003)24:3<287:RDRICC>2.0.ZU;2-Z
Fonte:
ISI
Lingua:
ENG
Soggetto:
HOMEOBOX GENE-EXPRESSION; SPINAL CORD PREPARATIONS; ONDINES-CURSE; DEVELOPMENTAL EXPRESSION; NEWBORN RATS; CHICK-EMBRYO; HOX11 GENE; BRAIN; MEDULLA; HINDBRAIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
27
Recensione:
Indirizzi per estratti:
Indirizzo: Korsmeyer, SJ Washington Univ, Sch Med, Dept Med, Howard Hughes Med Inst, St Louis, MO 63110 USA Washington Univ St Louis MO USA 63110 t Louis, MO 63110 USA
Citazione:
S. Shirasawa et al., "Rnx deficiency results in congenital central hypoventilation", NAT GENET, 24(3), 2000, pp. 287-290

Abstract

The genes Tlx1 (Hox11), Enx (Hox11L1, Tlx-2) and Rnx (Hox11L2, Tlx-3) constitute a family of orphan homeobox genes(1-10). In situ hybridization has revealed considerable overlap in their expression within the nervous system,but Rnx is singularly expressed in the developing dorsal and ventral region of the medulla oblongata. Tlx1-deficient and Enx-deficient mice display phenotypes in tissues where the mutated gene is singularly expressed, resulting in asplenogenesis(3,4) and hyperganglionic megacolon(8), respectively. To determine the developmental role of Rnx. we disrupted the locus in mouseembryonic stem (ES) cells. Rnx-deficient mice developed to term, but all died within 24 hours after birth from a central respiratory failure. The electromyographic activity of intercostal muscles coupled with the C4 ventral root activity assessed in a medulla-spinal cord preparation revealed a highrespiratory rate with short inspiratory duration and frequent apnea. Furthermore, a coordinate pattern existed between the abnormal activity of inspiratory neurons in the ventrolateral medulla and C4 motorneuron output, indicating a central respiratory defect in Rnx(-/-) mice. Thus, Rnx is criticalfor the development of the ventral medullary respiratory centre and its deficiency results in a syndrome resembling congenital central hypoventilation.

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Documento generato il 04/07/20 alle ore 21:01:19