Respiratory and autonomic dysfunction in congenital central hypoventilation syndrome

Autor(es)

Moreira, Thiago S. ; Takakura, Ana C. ; Czeisler, Catherine ; Otero, Jose J.

• Tipo de documento: Artigo de Revisão
• Fonte: Journal of Neurophysiology; v. 116, n. 2, p. 742-752, AUG 2016.
• Citações Web of Science: 9

Resumo

The developmental lineage of the PHOX2B-expressing neurons in the retrotrapezoid nucleus (RTN) has been extensively studied. These cells are thought to function as central respiratory chemoreceptors, i.e., the mechanism by which brain P-CO2 regulates breathing. The molecular and cellular basis of central respiratory chemoreception is based on the detection of CO2 via intrinsic proton receptors (TASK-2, GPR4) as well as synaptic input from peripheral chemoreceptors and other brain regions. Murine models of congenital central hypoventilation syndrome designed with PHOX2B mutations have suggested RTN neuron agenesis. In this review, we examine, through human and experimental animal models, how a restricted number of neurons that express the transcription factor PHOX2B play a crucial role in the control of breathing and autonomic regulation. (AU)

• Processo FAPESP: 13/10573-8 - Mecanismos neurais da superfície ventral do bulbo envolvidos na quimiorrecepção
• Beneficiário: Thiago dos Santos Moreira
• Linha de fomento: Auxílio à Pesquisa - Regular
• Processo FAPESP: 09/54888-7 - Mecanismos neurais envolvidos na quimiorrecepção
• Beneficiário: Eduardo Colombari
• Linha de fomento: Auxílio à Pesquisa - Temático
• Processo FAPESP: 14/22406-1 - Alterações respiratórias anatomofuncionais observadas em um modelo experimental da Doença de Parkinson
• Beneficiário: Ana Carolina Thomaz Takakura
• Linha de fomento: Auxílio à Pesquisa - Regular