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
en Zh
| WPRIM
| ID: wpr-345408
Biblioteca responsable:
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
Asunto principal:
Factores de Transcripción
/
Surfactantes Pulmonares
/
Transportadoras de Casetes de Unión a ATP
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Proteína C Asociada a Surfactante Pulmonar
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Proteínas de Unión al ADN
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Genética
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Homeostasis
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Enfermedades Pulmonares
/
Metabolismo
Límite:
Humans
Idioma:
Zh
Revista:
Chinese Journal of Medical Genetics
Año:
2016
Tipo del documento:
Article