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Published ahead of print on April 28, 2005, doi:10.1164/rccm.200411-1528OC

Am. J. Respir. Crit. Care Med., Volume 172, Number 2, July 2005, 238-243

A more recent version of this article appeared on July 15, 2005
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Submitted on November 16, 2004
Accepted on April 20, 2005

Sleep-disordered Breathing in Newborn Mice Heterozygous for the Transcription Factor Phox2b

Estelle Durand1, Stephane Dauger2, Alexandre Pattyn3, Claude Gaultier1, Christo Goridis3, and Jorge Gallego1*

1 U676, INSERM, Hopital Robert-Debre, Paris, France, 2 U676, INSERM, Hopital Robert-Debre, Paris, France; Service de Reanimation, Hopital Robert-Debre, Paris, France, 3 UMR 8542, CNRS, Ecole Normale Superieure, Paris, France

* To whom correspondence should be addressed. E-mail: gallego{at}rdebre.inserm.fr.

Rationale: Central Congenital Hypoventilation Syndrome (CCHS) is a rare autosomal dominant syndrome present from birth, and characterized by depressed ventilation during sleep. Heterozygous mutations of the homeobox gene Phox2b were recently found in a very high proportion of patients. Objectives: To determine whether newborn mice with heterozygous targeted deletion of the transcription factor Phox2b would display sleep-disordered breathing. Methods: We measured breathing pattern using whole-body plethysmography in wild-type and mutant five-day old mice, and we classified sleep-wake states using nuchal EMG and behavioral scores. Results: We found that sleep apnea total time was about 6 times longer (8.9±12 versus 1.5±2.2 seconds, p<0.0015), and ventilation during active sleep was 21% lower (18.4±5.1 versus 23.3±5.5 ml/g/s, p<0.006) in mutant than in wild-type pups. During wakefulness, apnea time and ventilation were not significantly different between mutant and wild-type pups. Mutant and wild-type pups showed highly similar sleep-wake states. Conclusion: Although their respiratory phenotype was much less severe than CCHS, the Phox2b+/- mutant mice showed sleep-disordered breathing which partially modeled the key feature of CCHS.
Key words: apnea, hypoventilation, Ondine syndrome




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