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Monoclonal Anti-LAMA2 antibody produced in mouse

产品编号:3147575
规格:clone 2D4, purified immunoglobulin, buffered aqueous solution
包装规格:100 μG
产品类别:进口试剂
品牌:Sigma-Aldrich
优惠价:立即咨询
产品价格
产品编号包装单位单价(元)国内现货国外库存询价单
3147575100 μG4470
产品别名

Monoclonal Anti-LAMA2 antibody produced in mouse

Anti-LAMM

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

基本信息
NACRES
NA.41
General description【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)
Immunogen【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
Biochem/physiol Actions【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.
Physical form【外形】
Solution in phosphate buffered saline, pH 7.4
Legal Information【法律信息】
GenBank is a registered trademark of United States Department of Health and Human Services
产品性质
Quality Level【质量水平】
100
biological source【生物来源】
mouse
conjugate【偶联物】
unconjugated
antibody form【抗体形式】
purified immunoglobulin
antibody product type
primary antibodies
clone【克隆】
2D4, monoclonal
form【形式】
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【同位素/亚型】
IgG1κ
GenBank® accession no.
NM_000426
UniProt accession no.【UniProt登记号】
P24043
shipped in【运输】
dry ice
storage temp.【储存温度】
−20℃
Gene Information
human ... LAMA2(3908)
产品说明
Disclaimer【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.
安全信息
Storage Class Code【储存分类代码】
12 - Non Combustible Liquids
Personal Protective Equipment【个人防护装备】
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
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