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WH0003908M1

Sigma-Aldrich

Monoclonal Anti-LAMA2 antibody produced in mouse

clone 2D4, purified immunoglobulin, buffered aqueous solution

Sinónimos:

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

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About This Item

Código UNSPSC:
12352203
NACRES:
NA.41

origen biológico

mouse

conjugado

unconjugated

forma del anticuerpo

purified immunoglobulin

tipo de anticuerpo

primary antibodies

clon

2D4, monoclonal

formulario

buffered aqueous solution

reactividad de especies

human

técnicas

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

isotipo

IgG1κ

Nº de acceso GenBank

Nº de acceso UniProt

Condiciones de envío

dry ice

temp. de almacenamiento

−20°C

modificación del objetivo postraduccional

unmodified

Información sobre el gen

human ... LAMA2(3908)

Descripción general

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)

Inmunógeno

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

Acciones bioquímicas o fisiológicas

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.

Forma física

Solution in phosphate buffered saline, pH 7.4

Información legal

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

Cláusula de descargo de responsabilidad

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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Opcional

Código de clase de almacenamiento

12 - Non Combustible Liquids

Punto de inflamabilidad (°F)

Not applicable

Punto de inflamabilidad (°C)

Not applicable

Equipo de protección personal

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


Certificados de análisis (COA)

Busque Certificados de análisis (COA) introduciendo el número de lote del producto. Los números de lote se encuentran en la etiqueta del producto después de las palabras «Lot» o «Batch»

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Visite la Librería de documentos

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

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