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INVITED REVIEW

HIGHLIGHTED TOPIC
Neural Control of Perinatal Respiration

Neural control of breathing: insights from genetic mouse models

¹Service de Physiologie, Hôpital Robert Debré, 75019 Paris, France; ²INSERM, U676, Hôpital Robert Debré, 75019 Paris, France; ³Université Denis Diderot, Paris, France

Recent studies described the in vivo ventilatory phenotype of mutant newborn mice with targeted deletions of genes involved in the organization and development of the respiratory-neuron network. Whole body flow barometric plethysmography is the noninvasive method of choice for studying unrestrained newborn mice. Breathing-pattern abnormalities with apneas occur in mutant newborn mice that lack genes involved in the development and modulation of rhythmogenesis. Studies of deficits in ventilatory responses to hypercapnia and/or hypoxia helped to identify genes involved in chemosensitivity to oxygen and carbon dioxide. Combined studies in mutant newborn mice and in humans have shed light on the pathogenesis of genetically determined respiratory-control abnormalities such as congenital central hypoventilation syndrome, Rett syndrome, and Prader-Willi syndrome. The development of mouse models has opened up the field of research into new treatments for respiratory-control disorders in humans.

newborn mice; whole body plethysmography; congenital central hypoventilation syndrome; Rett syndrome; Prader-Willi syndrome

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