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MABE230

Sigma-Aldrich

Anti-SMN2 Antibody, clone SMN-KH

clone SMN-KH, from mouse

Synonym(s):

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

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About This Item

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

biological source

mouse

Quality Level

100

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

SMN-KH, monoclonal

species reactivity

human

technique(s)

immunohistochemistry: suitable
western blot: suitable

isotype

IgG1κ

NCBI accession no.

NP_000335

UniProt accession no.

Q16637

shipped in

wet ice

target post-translational modification

unmodified

Gene Information

human ... SMN2(6607)

General description

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.

Immunogen

MBP-tagged recombinant protein corresponding to human SMN2.

Application

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.)

Quality

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.

Target description

~35 kDa observed

Physical form

Format: Purified

Other Notes

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

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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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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