Pulmonary surfactant homeostasis associated genetic abnormalities and lung diseases / 中华医学遗传学杂志
Chinese Journal of Medical Genetics
; (6): 564-568, 2016.
Article
em Zh
| WPRIM
| ID: wpr-345408
Biblioteca responsável:
WPRO
ABSTRACT
Pulmonary surfactant (PS) is synthesized and secreted by alveolar epithelial type II (AEII) cells, which is a complex compound formed by proteins and lipids. Surfactant participates in a range of physiological processes such as reducing the surface tension, keeping the balance of alveolar fluid, maintaining normal alveolar morphology and conducting host defense. Genetic disorders of the surfactant homeostasis genes may result in lack of surfactant or cytotoxicity, and lead to multiple lung diseases in neonates, children and adults, including neonatal respiratory distress syndrome, interstitial pneumonia, pulmonary alveolar proteinosis, and pulmonary fibrosis. This paper has provided a review for the functions and processes of pulmonary surfactant metabolism, as well as the connection between disorders of surfactant homeostasis genes and lung diseases.
Texto completo:
1
Índice:
WPRIM
Assunto principal:
Fatores de Transcrição
/
Surfactantes Pulmonares
/
Transportadores de Cassetes de Ligação de ATP
/
Proteína C Associada a Surfactante Pulmonar
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Proteínas de Ligação a DNA
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Genética
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Homeostase
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Pneumopatias
/
Metabolismo
Limite:
Humans
Idioma:
Zh
Revista:
Chinese Journal of Medical Genetics
Ano de publicação:
2016
Tipo de documento:
Article