WH0003908M1

Monoclonal Anti-LAMA2 antibody produced in mouse

clone 2D4, purified immunoglobulin, buffered aqueous solution

• Synonyme(s) : Anti-LAMM, Anti-laminin, alpha 2 (merosin, congenital muscular dystrophy)
• Code UNSPSC : 12352203
• Nomenclature NACRES : NA.41

Propriétés

• Source biologique: mouse
• Conjugué: unconjugated
• Forme d'anticorps: purified immunoglobulin
• Type de produit anticorps: primary antibodies
• Clone: 2D4, monoclonal
• Forme: buffered aqueous solution
• Espèces réactives: human
• Technique(s): immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable; indirect ELISA: suitable; western blot: 1-5 μg/mL
• Isotype: IgG1κ
• Numéro d'accès GenBank: NM_000426
• Numéro d'accès UniProt: P24043
• Conditions d'expédition: dry ice
• Température de stockage: −20°C
• Modification post-traductionnelle de la cible: unmodified
• Informations sur le gène: human ... LAMA2(3908)

Description

Description générale

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)

Immunogène

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

Actions biochimiques/physiologiques

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.

Forme physique

Solution in phosphate buffered saline, pH 7.4

Informations légales

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

Clause de non-responsabilité

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.

Informations sur la sécurité

• Code de la classe de stockage: 12 - Non Combustible Liquids
• Point d'éclair (°F): Not applicable
• Point d'éclair (°C): Not applicable
• Équipement de protection individuelle: Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)