生物
肾
肾脏发育
突变体
肺
器官发生
基底膜
整合素
形态发生
输尿管芽
细胞生物学
肾小球基底膜
受体
内分泌学
内科学
病理
肾小球肾炎
基因
遗传学
胚胎干细胞
医学
作者
Jordan A. Kreidberg,Michael J. Donovan,Stuart L. Goldstein,Helmut G. Rennke,Kenneth E. Shepherd,Rosemary Jones,Rudolf Jaenisch
出处
期刊:Development
[The Company of Biologists]
日期:1996-11-01
卷期号:122 (11): 3537-3547
被引量:713
标识
DOI:10.1242/dev.122.11.3537
摘要
ABSTRACT A mutation was targeted to the murine α3 integrin gene. Homozygous mutant mice survived to birth, but died during the neonatal period. The mutation caused abnormal kidney and lung development. Mutant kidneys displayed decreased branching of the medullary collecting ducts, although the number of nephrons was not altered. Proximal tubules exhibited two distinct subsets of abnormalities, with the epithelial cells either containing excess lysosomes or becoming microcystic. In addition, glomerular development was markedly affected. In mutant kidneys, the extent of branching of glomerular capillary loops was decreased, with capillary lumina being wider than normal. The glomerular basement membrane was disorganized and glomerular podocytes were unable to form mature foot processes. Branching of the bronchi in lungs of mutant mice was also decreased and the large bronchi extended to the periphery. These results indicate a role for integrin receptors in basement membrane organization and branching morphogenesis.
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