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肺表面活性蛋白C(surfactant protein C,SP-C)是肺表面活性物质组成成分之一,其基因仅定位于Ⅱ型肺泡上皮细胞(ATII)中。编码SP-C的突变与新生儿及儿童呼吸窘迫综合征和肺间质性疾病(ILD)密切相关[14]。SP-C蛋白质的表达受发育和基因等的调节,随着胎龄的增加而增多,并对出生时肺表面活性物质功能起关键作用。SP-C在肺泡II型上皮细胞合成,包装成层状结构后进入肺泡表面细胞中,它的功能是稳定表面活性物质磷脂,使其沿肺泡表面分泌和依附[15]。目前SP-B遗传缺陷在RDS的作用机制逐步明朗化,而对SP-C遗传缺陷研究较少,本文就SP-C的生物学特性、调节因子、遗传缺陷的临床表现、各种遗传缺陷的组织病理学和超微结构特征、以及遗传缺陷的免疫组织化学和发病机制做一综述,以期加深人们对SP-C的认识。
The surfactant protein C (SP-C) is one of the components of pulmonary surfactant. Its gene is only located in type A alveolar epithelial cells (ATII). Mutations encoding SP-C are closely related to neonatal and childhood respiratory distress syndrome and interstitial lung disease (ILD) [14]. SP-C protein expression by the development and gene regulation, with the increase of gestational age increased, and play a key role in birth pulmonary surfactant function. SP-C is synthesized in alveolar type II epithelial cells and packaged into lamellar structures into cells on the alveolar surface. Its function is to stabilize the surface-active substance phospholipid and make it secrete and adhere along the alveolar surface [15]. At present, the mechanism of SP-B genetic defects in RDS is gradually clarified, but there are few studies on the genetic defects of SP-C. In this paper, the biological characteristics of SP-C, regulatory factors, the clinical manifestations of genetic defects, Histopathology and ultrastructural features, as well as the genetic defects of immunohistochemistry and pathogenesis make a review, with a view to deepen people’s understanding of SP-C.