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Merck
CN

WH0003908M1

Monoclonal Anti-LAMA2 antibody produced in mouse

clone 2D4, purified immunoglobulin, buffered aqueous solution

Synonym(s):

Anti-LAMM, Anti-laminin, alpha 2 (merosin, congenital muscular dystrophy)

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About This Item

NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
2D4, monoclonal
Application:
ELISA (i), IHC (p), WB
Citations:
2
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biological source

mouse

conjugate

unconjugated

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

2D4, monoclonal

form

buffered aqueous solution

species reactivity

human

technique(s)

immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable, indirect ELISA: suitable, western blot: 1-5 μg/mL

isotype

IgG1κ

GenBank accession no.

UniProt accession no.

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... LAMA2(3908)

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General description

Laminin, an extracellular protein, is a major component of the basement membrane. It is thought to mediate the attachment, migration, and organization of cells into tissues during embryonic development by interacting with other extracellular matrix components. It is composed of three subunits, alpha, beta, and gamma, which are bound to each other by disulfide bonds into a cross-shaped molecule. This gene encodes the alpha 2 chain, which constitutes one of the subunits of laminin 2 (merosin) and laminin 4 (s-merosin). Mutations in this gene have been identified as the cause of congenital merosin-deficient muscular dystrophy. Two transcript variants encoding different proteins have been found for this gene. (provided by RefSeq)

Immunogen

LAMA2 (NP_000417, 3013 a.a. ~ 3122 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
DAGVPGHLCDGQWHKVTANKIKHRIELTVDGNQVEAQSPNPASTSADTNDPVFVGGFPDDLKQFGLTTSIPFRGCIRSLKLTKGTGKPLEVNFAKALELRGVQPVSCPAN

Biochem/physiol Actions

Laminins are glycoproteins that function in extracellular matrix architecture, cell adhesion, differentiation, and neurite outgrowth. Mutations in the gene LAMA2 (laminin-2) leading to either partial deficiency or complete absence of laminin α2 have been associated with limb-girdle muscular dystrophy (LGMD), characterized by severe, early-onset congenital muscular dystrophy to mild, later childhood-onset limb-girdle type muscular dystrophy, hypotonia with muscle weakness at early infancy, poor spontaneous movements with contractures of the large joints, and weak cry along with respiratory dysfunction.

Physical form

Solution in phosphate buffered saline, pH 7.4

Legal Information

GenBank is a registered trademark of United States Department of Health and Human Services

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Storage Class

12 - Non Combustible Liquids

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)

Regulatory Information

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LAMA2-Related Muscular Dystrophy.
Quijano-Roy S
GeneReviews(?) (2012)
Clinical and molecular characterization of limb-girdle muscular dystrophy due to LAMA2 mutations.
Gavassini BF
Muscle and Nerve, 44, 703-709 (2011)

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