title: The renal glomerulus of mice lacking s-laminin/laminin beta 2: nephrosis despite molecular compensation by laminin beta 1.
abstract: S-laminin/laminin beta 2, a homologue of the widely distributed laminin B1/beta 1 chain, is a major component of adult renal glomerular basement membrane (GBM). Immature GBM bears beta 1, which is replaced by beta 2 as development proceeds. In mutant mice that lack beta 2, the GBM remains rich in beta 1, suggesting that a feedback mechanism normally regulates GBM maturation. The beta 2-deficient GBM is structurally intact and contains normal complements of several collagenous and noncollagenous glycoproteins. However, mutant mice develop massive proteinuria due to failure of the glomerular filtration barrier. These results support the idea that laminin beta chains are functionally distinct although they assemble to form similar structures. Laminin beta 2-deficient mice may provide a model for human congenital or idiopathic nephrotic syndromes.
authors.affiliation: Department of Anatomy, Washington University School of Medicine, St. Louis, Missouri 63110, USA.
authors.forename: P G
authors.initials: PG
authors.lastname: Noakes
authors.forename: J H
authors.initials: JH
authors.lastname: Miner
authors.forename: M
authors.initials: M
authors.lastname: Gautam
authors.forename: J M
authors.initials: JM
authors.lastname: Cunningham
authors.forename: J R
authors.initials: JR
authors.lastname: Sanes
authors.forename: J P
authors.initials: JP
authors.lastname: Merlie
mesh_terms: D000818:Animals; D001485:Basement Membrane / Q000473:pathology; D004195:Disease Models, Animal; D007678:Kidney Glomerulus / Q000378:metabolism* / Q000473:pathology; D007797:Laminin / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D051379:Mice; D018345:Mice, Knockout; D009401:Nephrosis / Q000235:genetics / Q000378:metabolism* / Q000473:pathology
chemical_list: D007797:Laminin; C058783:laminin beta2