Disease relevance of Lama5

• Targeted mutation of the mouse laminin alpha 5 gene Lama5 causes embryonic lethality at E14-E17 associated with exencephaly, syndactyly, placentopathy, and kidney defects, all attributable to abnormal basement membranes [1].

High impact information on Lama5

• Lama5-null mice develop a small tooth germ with defective cusp formation and have reduced proliferation of dental epithelium [2].
• Here, we have examined the role of laminin alpha5 (Lama5) in tooth development using laminin alpha5-null mouse primary dental epithelium and tooth germ organ cultures [2].
• A new mouse PKD model has been produced through insertion of a PGKneo cassette in an intron of the gene that encodes laminin alpha5 (Lama5), a major tubular and glomerular basement membrane component that is important for glomerulogenesis and ureteric bud branching [3].
• Crossing the transgenes onto the Lama5-/- background generates "pseudo-knockins", so called because endogenous laminin alpha5 is replaced by transgene-encoded proteins [4].
• The mouse laminin alpha5 chain gene (Lama5) has been mapped close to the locus of the semidominant ragged (Ra) mutation on distal chromosome 2 [5].

Associations of Lama5 with chemical compounds

• Inhibition of laminin-511 receptor beta1 integrin disrupted DP primary cilia formation as well as hair development [6].

References