Laminin-11.
Laminins are a family of glycoproteins which are ubiquitous components of basement membranes and play key structural and functional roles. Eleven isoforms have been identified to date; each is an alpha beta gamma heterotrimer assembled from a repertoire of five alpha, three beta and two gamma chains. Studies of laminin-11 (alpha 5 beta 2 gamma 1) illustrate the unique expression patterns and distinct functions that can be displayed by laminin isoforms. Laminin-11 is found in the glomerular basement membrane in kidney, in the neuromuscular synaptic cleft in skeletal muscle and in other tissues such as placenta and lung. Mice lacking laminin-11 exhibit defective glomerular filtration and developmental defects in neuromuscular synapse formation, with Schwann cells invading the synaptic cleft. Consistent with these observations, both motoneurons and Schwann cells distinguish laminin-11 from other isoforms in vitro. These results suggest that laminin-11 is a structural component of the basement membrane which influences cell behavior in physiologically relevant ways. A greater understanding of laminin-11 assembly and basement membrane incorporation could provide a logical basis for therapy in merosin-deficient congenital muscular dystrophy.
Miner
J H
JH
Department of Medicine/Renal Division, Washington University School of Medicine, St. Louis, MO 63110, USA. minerj@thalamus.wustl.edu
Patton
B L
BL
D000818:Animals; D001485:Basement Membrane / Q000737:chemistry; D007797:Laminin / Q000737:chemistry* / Q000235:genetics; D009136:Muscular Dystrophies / Q000235:genetics / Q000628:therapy; D009469:Neuromuscular Junction / Q000378:metabolism; D011487:Protein Conformation; D020033:Protein Isoforms / Q000737:chemistry
D007797:Laminin; D020033:Protein Isoforms