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Merck
모든 사진(1)

Key Documents

MABE230

Sigma-Aldrich

Anti-SMN2 Antibody, clone SMN-KH

clone SMN-KH, from mouse

동의어(들):

Survival motor neuron protein, Component of gems 1, Gemin-1

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About This Item

UNSPSC 코드:
12352203
eCl@ss:
32160702
NACRES:
NA.41

생물학적 소스

mouse

Quality Level

항체 형태

purified immunoglobulin

항체 생산 유형

primary antibodies

클론

SMN-KH, monoclonal

종 반응성

human

기술

immunohistochemistry: suitable
western blot: suitable

동형

IgG1κ

NCBI 수납 번호

UniProt 수납 번호

배송 상태

wet ice

타겟 번역 후 변형

unmodified

유전자 정보

human ... SMN2(6607)

일반 설명

Survival of Motor Neuron (SMN) is expressed from two linked paralogous genes, SMN1 and SMN2. SMNs are primarily localized in the cytoplasm and nuclear gems of all cells, where they are understood to mediate the assembly of spliceosomal small nuclear ribonucleoprotein particles (snRNPs). They also play a role in the metabolism of snoRNPs and are required for the splicing of pre-mRNA in the nucleus. The progressive loss of functional SMNs in the anterior horn of the spinal cord is a critical cause of Spinal Muscular Atrophy (SMA), an autosomal recessive neuromuscular disease. There are three known types of childhood-onset SMA, in addition to a fourth type that is characterized by adult-onset SMA.

면역원

MBP-tagged recombinant protein corresponding to human SMN2.

애플리케이션

Anti-SMN2 Antibody, clone SMN-KH is a Mouse Monoclonal Antibody for detection of SMN2 also known as Survival motor neuron protein & has been validated in WB & IHC.
Western Blot Analysis: A representative lot from an independent laboratory detected SMN2 in HeLa cell lysate.

Immunohistochemistry Analysis: A respresentative lot from an independent laboratory detected human SMN2 in SMA type III mouse model thoracic spinal cord tissue. (Hua, Y., et al. (2010). Genes Dev. 24(15):1634-1644.)

품질

Evaluated by Western Blot in HeLa cell lysate.

Western Blot Analysis: 0.5 µg/mL of this antibody detected SMN2 in 10 µg of HeLa cell lysate.

표적 설명

~35 kDa observed

물리적 형태

Format: Purified

기타 정보

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.

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Storage Class Code

12 - Non Combustible Liquids

WGK

WGK 1

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable


시험 성적서(COA)

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문서 라이브러리 방문

Antisense correction of SMN2 splicing in the CNS rescues necrosis in a type III SMA mouse model.
Hua, Yimin, et al.
Genes & Development, 24, 1634-1644 (2010)
Anton J Blatnik et al.
Human molecular genetics, 29(21), 3477-3492 (2020-10-20)
Spinal muscular atrophy (SMA) is caused by mutation or deletion of survival motor neuron 1 (SMN1) and retention of SMN2 leading to SMN protein deficiency. We developed an immortalized mouse embryonic fibroblast (iMEF) line in which full-length wild-type Smn (flwt-Smn)

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