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Merck
모든 사진(3)

주요 문서

WH0003908M1

Sigma-Aldrich

Monoclonal Anti-LAMA2 antibody produced in mouse

clone 2D4, purified immunoglobulin, buffered aqueous solution

동의어(들):

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

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About This Item

UNSPSC 코드:
12352203
NACRES:
NA.41
가격 및 재고 정보를 현재 이용할 수 없음 고객지원팀으로 연락바랍니다.

생물학적 소스

mouse

결합

unconjugated

항체 형태

purified immunoglobulin

항체 생산 유형

primary antibodies

클론

2D4, monoclonal

양식

buffered aqueous solution

종 반응성

human

기술

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

동형

IgG1κ

GenBank 수납 번호

UniProt 수납 번호

배송 상태

dry ice

저장 온도

−20°C

타겟 번역 후 변형

unmodified

유전자 정보

human ... LAMA2(3908)

일반 설명

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)

면역원

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

생화학적/생리학적 작용

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.

물리적 형태

Solution in phosphate buffered saline, pH 7.4

법적 정보

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

면책조항

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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Storage Class Code

12 - Non Combustible Liquids

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable

개인 보호 장비

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


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문서 라이브러리 방문

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)

질문

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