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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) |