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Novocastra Merosin Laminin Alpha 2 Chain

Antigen Background

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.

Human skeletal muscle: immunohistochemical staining for merosin using NCL-MEROSIN.  Note membrane staining of normal muscle fibers (A) and absence of staining of muscle fibers in an individual with chromosome 6-linked congenital muscular dystrophy (B). Frozen sections. Photographs supplied courtesy of Dr Louise V B Anderson.
Human skeletal muscle: immunohistochemical staining for merosin using NCL-MEROSIN. Note membrane staining of normal muscle fibers (A) and absence of staining of muscle fibers in an individual with chromosome 6-linked congenital muscular dystrophy (B). Frozen sections. Photographs supplied courtesy of Dr Louise V B Anderson.

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CLONE: MER3/22B2

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Code Name Configuration Use Datasheet MSDS Qty
MEROSIN-CE 1ml NCL-MEROSIN Lyophilised Concentrated Monoclonal Antibody F Info MSDS
MEROSIN-CE-S 0.1ml NCL-MEROSIN Lyophilised Concentrated Monoclonal Antibody F Info MSDS
MEROSIN 1ml NCL-MEROSIN Lyophilised Concentrated Monoclonal Antibody F Info MSDS