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主要文件

安全信息

WH0003908M1

Sigma-Aldrich

Monoclonal Anti-LAMA2 antibody produced in mouse

clone 2D4, purified immunoglobulin, buffered aqueous solution

别名:

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

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About This Item

UNSPSC代码:
12352203
NACRES:
NA.41

生物来源

mouse

偶联物

unconjugated

抗体形式

purified immunoglobulin

抗体产品类型

primary antibodies

克隆

2D4, monoclonal

表单

buffered aqueous solution

种属反应性

human

技术

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

同位素/亚型

IgG1κ

GenBank登记号

UniProt登记号

运输

dry ice

储存温度

−20°C

靶向翻译后修饰

unmodified

基因信息

human ... LAMA2(3908)

一般描述

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)

免疫原

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

生化/生理作用

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.

外形

Solution in phosphate buffered saline, pH 7.4

法律信息

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

免责声明

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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储存分类代码

12 - Non Combustible Liquids

闪点(°F)

Not applicable

闪点(°C)

Not applicable

个人防护装备

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

法规信息

新产品

历史批次信息供参考:

分析证书(COA)

Lot/Batch Number

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访问文档库

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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