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基底膜的结构与功能。

Structure and function of basement membranes.

作者信息

Engvall E

机构信息

Department of Developmental Biology, The Wenner-Gren Institute, Stockholm University, Sweden.

出版信息

Int J Dev Biol. 1995 Oct;39(5):781-7.

PMID:8645562
Abstract

The importance of basement membranes in development and adult tissue function has been inferred from a number of observations. Cells migrate along basement membranes during development, basement membranes are required for the polarization of cells in both the embryo and the adult, and basement membranes serve as substrates for cell adhesion and migration during wound healing and nerve regeneration. The importance of basement membranes in adult tissue function has been directly demonstrated by the genetic diseases caused by mutations in the genes for structural basement membrane components. Examples of such diseases are Alport syndrome and junctional epidermolysis bullosa. Recently, defects in the major laminin variant in muscle, merosin, has been shown to be correlated with muscular dystrophies in man and animals. We are using the dystrophic mutant mouse dy, which lacks laminin-2, to analyze the function of laminin-2 in different tissues. Studies of laminin defects in animals and humans are expected to give new information on the function of basement membrane in general and on laminin in particular. Such information may give directions for future diagnosis and treatment of diseases involving basement membranes.

摘要

基底膜在发育和成年组织功能中的重要性已从多项观察中推断得出。在发育过程中,细胞沿着基底膜迁移,胚胎和成年个体中细胞的极化都需要基底膜,并且在伤口愈合和神经再生过程中,基底膜作为细胞黏附和迁移的底物。基底膜在成年组织功能中的重要性已通过由基底膜结构成分基因突变引起的遗传疾病得到直接证明。此类疾病的例子有阿尔波特综合征和交界性大疱性表皮松解症。最近,已表明肌肉中主要的层粘连蛋白变体——巢蛋白的缺陷与人类和动物的肌肉营养不良有关。我们正在使用缺乏层粘连蛋白 -2 的营养不良突变小鼠dy来分析层粘连蛋白 -2 在不同组织中的功能。对动物和人类层粘连蛋白缺陷的研究有望提供关于基底膜总体功能,尤其是层粘连蛋白功能的新信息。这些信息可能为未来涉及基底膜疾病的诊断和治疗指明方向。

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