Severe Fibronectin-Deposit Renal Glomerular Disease in Mice Lacking Uteroglobin

Despite myriads of biological activities ascribed to uteroglobin (UG), a steroid-inducible secreted protein, its physiological functions are unknown. Mice in which the uteroglobin gene was disrupted had severe renal disease that was associated with massive glomerular deposition of predominantly mult...

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Bibliographic Details
Published in:Science (American Association for the Advancement of Science) 1997-05, Vol.276 (5317), p.1408-1412
Main Authors: Zhang, Zhongjian, Kundu, Gopal C., Yuan, Chiun-Jye, Ward, Jerrold M., Lee, Eric J., DeMayo, Francesco, Westphal, Heiner, Mukherjee, Anil B.
Format: Article
Language:English
Subjects:
Animals
Antibodies
Biological and medical sciences
Cells, Cultured
Classical genetics, quantitative genetics, hybrids
Collagens
Crosses, Genetic
Disease
Epithelial cells
Fibronectins
Fibronectins - metabolism
Fundamental and applied biological sciences. Psychology
Gene Targeting
Genetic loci
Genetics of eukaryotes. Biological and molecular evolution
Humans
Inhibition
Kidney diseases
Kidney Diseases - embryology
Kidney Diseases - genetics
Kidney Diseases - pathology
Kidney Glomerulus - embryology
Kidney Glomerulus - metabolism
Kidney Glomerulus - ultrastructure
Kidneys
Liver
Lungs
Messenger RNA
Mice
Mice, Inbred C57BL
Physiological aspects
Polymerase chain reaction
Proteins
Rodents
Uteroglobin - deficiency
Uteroglobin - genetics
Uteroglobin - physiology
Uteroglobulin
Vertebrata
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Description
Summary:Despite myriads of biological activities ascribed to uteroglobin (UG), a steroid-inducible secreted protein, its physiological functions are unknown. Mice in which the uteroglobin gene was disrupted had severe renal disease that was associated with massive glomerular deposition of predominantly multimeric fibronectin (Fn). The molecular mechanism that normally prevents Fn deposition appears to involve high-affinity binding of UG with Fn to form Fn-UG heteromers that counteract Fn self-aggregation, which is required for abnormal tissue deposition. Thus, UG is essential for maintaining normal renal function in mice, which raises the possibility that an analogous pathogenic mechanism may underlie genetic Fn-deposit human glomerular disease.
ISSN:0036-8075
1095-9203
DOI:10.1126/science.276.5317.1408