TY - JOUR
T1 - Normal ventilation and ventilatory responses to chemical stimuli in juvenile mutant mice deficient in endothelin-3
AU - Nakamura, Akira
AU - Kuwaki, Tomoyuki
AU - Kuriyama, Takayuki
AU - Yanagisawa, Masashi
AU - Fukuda, Yasuichiro
N1 - Funding Information:
We thank Sahar Seyedkalal, Chikako Kumagai and Wataru Nakamura for the technical assistance. Part of this work was supported by Grants-in Aid for Scientific Research from the Ministry of Education, Science, Culture and Sports, Japan and Grants from the Naito Foundation, the Takeda Science Foundation, the Perot Family Foundation and the W.M. Keck Foundation.
PY - 2000
Y1 - 2000
N2 - Congenital central hypoventilation syndrome (CCHS) and Hirschsprung's disease (HSCR) are often classified as neurocristopathies and are thought to share a common molecular pathogenesis related to the genes that control the development of neural crest cells. We examined whether endothelin-3 (ET-3), one of the developmental regulators of neural crest cells and of which null mutation results in aganglionic megacolon in mice, fulfills the requirements for such a common molecule. To investigate the possible involvement of ET-3 in central ventilatory control, we measured ventilation in mutant mice deficient in ET-3 by whole body plethysmography. Tidal volume and breathing frequency were measured during breathing of room air, hypoxic, hyperoxic, or hypercapnic gas mixtures in awake and anesthetized mice. There were no significant differences in resting ventilation as well as ventilatory responses to hypoxia and hypercapnia between ET-3-knockout mice and wild-type mice. Our results indicate that ET-3 can not be considered as a common pathogenic mechanism for CCHS and HSCR at least in mice. (C) 2000 Elsevier Science B.V.
AB - Congenital central hypoventilation syndrome (CCHS) and Hirschsprung's disease (HSCR) are often classified as neurocristopathies and are thought to share a common molecular pathogenesis related to the genes that control the development of neural crest cells. We examined whether endothelin-3 (ET-3), one of the developmental regulators of neural crest cells and of which null mutation results in aganglionic megacolon in mice, fulfills the requirements for such a common molecule. To investigate the possible involvement of ET-3 in central ventilatory control, we measured ventilation in mutant mice deficient in ET-3 by whole body plethysmography. Tidal volume and breathing frequency were measured during breathing of room air, hypoxic, hyperoxic, or hypercapnic gas mixtures in awake and anesthetized mice. There were no significant differences in resting ventilation as well as ventilatory responses to hypoxia and hypercapnia between ET-3-knockout mice and wild-type mice. Our results indicate that ET-3 can not be considered as a common pathogenic mechanism for CCHS and HSCR at least in mice. (C) 2000 Elsevier Science B.V.
KW - Control of breathing, central
KW - Development, neural crest
KW - Disease, Hirschsprung
KW - Disease, congenital central hypoventilation syndrome
KW - Mammals, mouse
KW - Mediators, endothelin-3
KW - Mutation, endothelin-3
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U2 - 10.1016/S0034-5687(00)00181-X
DO - 10.1016/S0034-5687(00)00181-X
M3 - Article
C2 - 11084198
AN - SCOPUS:0033751353
SN - 0034-5687
VL - 124
SP - 1
EP - 9
JO - Respiration Physiology
JF - Respiration Physiology
IS - 1
ER -