Novocastra Merosin Laminin Alpha 2 Chain
The dystrophin-glycoprotein complex is localized to the muscle membrane. Several members of this complex are reported to be implicated in muscular dystrophy. Dystrophin expression is altered in Duchenne and Becker muscular dystrophy and four types of limb girdle muscular dystrophy are caused by mutations in the genes for alpha, beta, gamma and delta-sarcoglycan.An extracellular member of this complex is alpha-dystroglycan and linked to this, in the extracellular matrix, is laminin. The muscle specific form of laminin, merosin, is composed of three chains: alpha 2, beta 1 and gamma 1. Mutations in the chromosome 6 encoded gene for the laminin alpha 2 chain of merosin are responsible for a form of congenital muscular dystrophy (CMD). Merosin negative CMD is characterized by a severe clinical phenotype and is associated with white matter changes on brain imaging.
Product Specific Information
NCL-MEROSIN reacts with the 300 kD fragment of merosin (Sewry et al. Muscle and Nerve Supplement. 7, S109: (1998)) labeling with an antibody to beta-spectrin to monitor membrane integrity, is an essential immunohistochemical control.