Human surfactant protein B: structure, function, regulation, and genetic disease.
Human surfactant protein B: structure, function, regulation, and genetic disease.
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DOI：
10.1152/physrev.1995.75.4.749
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发表时间：
1995-10
影响因子：
33.6
通讯作者：
Jeffrey A. Whitsett;L. Nogee;Timothy E. Weaver;A. Horowitz
中科院分区：
文献类型：
--
作者：
Jeffrey A. Whitsett;L. Nogee;Timothy E. Weaver;A. Horowitz
疏水性表面活性蛋白（SP-B）和SP-B基因的结构和功能的阐明提供了关键的洞察表面活性剂的稳态和控制呼吸上皮细胞基因表达。表面活性剂蛋白B与表面活性剂蛋白A（SP-A）、表面活性剂蛋白C（SP-C）和表面活性剂磷脂一起，有助于表面活性剂颗粒的结构和功能，决定表面活性剂磷脂和蛋白质被II型上皮细胞加工、路由、包装和从板层体分泌的表面活性和途径。分泌后，SP-B在决定管状髓鞘的结构、扩散的稳定性和快速性以及表面活性剂磷脂的回收方面发挥着至关重要的作用。肺泡表面活性物质的体内平衡的生化和结构信号可能是介导的表面活性物质蛋白和磷脂之间的相互作用，产生离散的结构形式，不同的大小，前蛋白和磷脂含量。结构、蛋白质和大小的差异可能决定表面活性剂颗粒的功能、它们的催化作用或肺泡巨噬细胞和气道上皮细胞的再循环。对SP-B基因的遗传控制的分析导致了DNA-蛋白质相互作用的定义，其通常决定呼吸道上皮细胞基因表达。SP-B在肺功能中的重要作用是通过对由人类SP-B基因突变引起的致死性新生儿呼吸道疾病-遗传性SP-B缺乏症的研究确定的。
Elucidation of the structure and function of the hydrophobic surfactant protein (SP-B) and the SP-B gene has provided critical insight into surfactant homeostasis and control of respiratory epithelial cell gene expression. Surfactant protein B, in concert with surfactant protein A (SP-A), surfactant protein C (SP-C), and surfactant phospholipids, contributes to the structure and function of surfactant particles, determining surface activities and pathways by which surfactant phospholipids and proteins are processed, routed, packaged, and secreted from lamellar bodies by type II epithelial cells. After secretion, SP-B plays an essential role in determining the structure of tubular myelin, the stability and rapidity of spreading, and the recycling of surfactant phospholipids. The biochemical and structural signals underlying the homeostasis of alveolar surfactant are likely mediated by interactions between the surfactant proteins and phospholipids producing discrete structural forms that vary in size, aproprotein, and phospholipid content. Distinctions in structure, protein, and size are likely to determine the function of surfactant particles, their catabolism, or recycling by alveolar macrophages and airway epithelial cells. Analysis of the genetic controls governing the SP-B gene has led to the definition of DNA-protein interactions that determine respiratory epithelial cell gene expression in general. The important role of SP-B in lung function was defined by the study of a lethal neonatal respiratory disease, hereditary SP-B deficiency, caused by mutations in the human SP-B gene.