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Defective forebrain development in mice lacking gp330/megalin

Item Type:Article
Title:Defective forebrain development in mice lacking gp330/megalin
Creators Name:Willnow, T.E. and Hilpert, J. and Armstrong, S.A. and Rohlmann, A. and Hammer, R.E. and Burns, D.K. and Herz, J.
Abstract:gp330/megalin, a member of the low density lipoprotein (LDL) receptor gene family, is expressed on the apical surfaces of epithelial tissues, including the neuroepithelium, where it mediates the endocytic uptake of diverse macromolecules, such as cholesterol-carrying lipoproteins, proteases, and antiproteinases. Megalin knockout mice manifest abnormalities in epithelial tissues including lung and kidney that normally express the protein and they die perinatally from respiratory insufficiency. In brain, impaired proliferation of neuroepithelium produces a holoprosencephalic syndrome, characterized by lack of olfactory bulbs, forebrain fusion, and a common ventricular system. Similar syndromes in humans and animals are caused by insufficient supply of cholesterol during development. Because megalin can bind lipoproteins, we propose that the receptor is part of the maternal-fetal lipoprotein transport system and mediates the endocytic uptake of essential nutrients in the postgastrulation stage.
Keywords:Brain, Cholesterol, Developmental Gene Expression Regulation, Heymann Nephritis Antigenic Complex, Holoprosencephaly, Kidney, Membrane Glycoproteins, Electron, Scanning Microscopy, Messenger RNA, LDL Receptors, Animals, Mice
Source:Proceedings of the National Academy of Sciences of the United States of America
ISSN:0027-8424
Publisher:National Academy of Sciences (U.S.A.)
Volume:93
Number:16
Page Range:8460-8464
Date:6 August 1996
Official Publication:http://www.pnas.org/content/93/16/8460.abstract
PubMed:View item in PubMed

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