title: Genetic inactivation of the laminin alpha5 chain receptor Lu/BCAM leads to kidney and intestinal abnormalities in the mouse.
abstract: Lutheran blood group and basal cell adhesion molecule (Lu/BCAM) has been recognized as a unique receptor for laminin alpha5 chain in human red blood cells and as a coreceptor in epithelial, endothelial, and smooth muscle cells. Because limited information is available regarding the function of this adhesion glycoprotein in vivo, we generated Lu/BCAM-null mice and looked for abnormalities in red blood cells as well as in kidney and intestine, two tissues showing alteration in laminin alpha5 chain-deficient mice. We first showed that, in contrast to humans, wild-type murine red blood cells failed to express Lu/BCAM. Lu/BCAM-null mice were healthy and developed normally. However, although no alteration of the renal function was evidenced, up to 90% of the glomeruli from mutant kidneys exhibited abnormalities characterized by a reduced number of visible capillary lumens and irregular thickening of the glomerular basement membrane. Similarly, intestine analysis of mutant mice revealed smooth muscle coat thickening and disorganization. Because glomerular basement membrane and smooth muscle coat express laminin alpha5 chain and are in contact with cell types expressing Lu/BCAM in wild-type mice, these results provide evidence that Lu/BCAM, as a laminin receptor, is involved in vivo in the maintenance of normal basement membrane organization in the kidney and intestine.
authors.affiliation: Institut National de la Santé et de la Recherche Médicale, Unité 665, Institut National de la Transfusion Sanguine, Paris, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Rahuel
authors.forename: Anne
authors.initials: A
authors.lastname: Filipe
authors.forename: Léa
authors.initials: L
authors.lastname: Ritie
authors.forename: Wassim
authors.initials: W
authors.lastname: El Nemer
authors.forename: Natacha
authors.initials: N
authors.lastname: Patey-Mariaud
authors.forename: Dominique
authors.initials: D
authors.lastname: Eladari
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Cartron
authors.forename: Patricia
authors.initials: P
authors.lastname: Simon-Assmann
authors.forename: Caroline
authors.initials: C
authors.lastname: Le Van Kim
authors.forename: Yves
authors.initials: Y
authors.lastname: Colin
mesh_terms: D000818:Animals; D001485:Basement Membrane / Q000002:abnormalities* / Q000378:metabolism / Q000648:ultrastructure; D015815:Cell Adhesion Molecules; D042783:Endothelial Cells / Q000378:metabolism; D004912:Erythrocytes / Q000378:metabolism; D005260:Female; D015870:Gene Expression; D050533:Glomerular Basement Membrane / Q000002:abnormalities / Q000378:metabolism / Q000648:ultrastructure; D007413:Intestinal Mucosa / Q000378:metabolism; D007422:Intestines / Q000002:abnormalities*; D007668:Kidney / Q000002:abnormalities* / Q000378:metabolism; D007677:Kidney Function Tests; D007678:Kidney Glomerulus / Q000002:abnormalities / Q000648:ultrastructure; D007797:Laminin / Q000032:analysis / Q000378:metabolism*; D008188:Lutheran Blood-Group System; D008297:Male; D008562:Membrane Glycoproteins / Q000235:genetics* / Q000378:metabolism; D050527:Mesangial Cells / Q000378:metabolism; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D008854:Microscopy, Electron; D009130:Muscle, Smooth / Q000002:abnormalities / Q000648:ultrastructure; D032389:Myocytes, Smooth Muscle / Q000378:metabolism; D050199:Podocytes / Q000378:metabolism; D017474:Receptors, Laminin / Q000378:metabolism
chemical_list: C477645:Bcam protein, mouse; D015815:Cell Adhesion Molecules; D007797:Laminin; D008188:Lutheran Blood-Group System; D008562:Membrane Glycoproteins; D017474:Receptors, Laminin; C097043:laminin alpha5