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05-1532

Sigma-Aldrich

Anti-SMN Antibody, clone 2B1

clone 2B1, from mouse

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Synonym(s):
Component of gems 1, gemin 1, spinal muscular atrophy (Werdnig-Hoffmann disease, Kugelberg-Welander disease), survival of motor neuron 1, telomeric
eCl@ss:
32160702
NACRES:
NA.41

biological source

mouse

Quality Level

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

2B1, monoclonal

species reactivity

human

species reactivity (predicted by homology)

Xenopus, mouse

technique(s)

immunocytochemistry: suitable
immunoprecipitation (IP): suitable
western blot: suitable

isotype

IgG1κ

NCBI accession no.

UniProt accession no.

shipped in

wet ice

target post-translational modification

unmodified

Gene Information

human ... SMA4(11039)

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This Item
04-1466MABE23005-1540-M
Anti-SMN Antibody, clone 2B1 clone 2B1, from mouse

05-1532

Anti-SMN Antibody, clone 2B1

Anti-SMN Antibody, clone 62E7 clone 62E7, from mouse

04-1466

Anti-SMN Antibody, clone 62E7

Anti-SMN2 Antibody, clone SMN-KH clone SMN-KH, from mouse

MABE230

Anti-SMN2 Antibody, clone SMN-KH

biological source

mouse

biological source

mouse

biological source

mouse

biological source

mouse

antibody form

purified immunoglobulin

antibody form

purified immunoglobulin

antibody form

purified immunoglobulin

antibody form

purified antibody

clone

2B1, monoclonal

clone

62E7, monoclonal

clone

SMN-KH, monoclonal

clone

2E17, monoclonal

technique(s)

immunocytochemistry: suitable, western blot: suitable, immunoprecipitation (IP): suitable

technique(s)

immunocytochemistry: suitable, immunoprecipitation (IP): suitable, western blot: suitable

technique(s)

immunohistochemistry: suitable, western blot: suitable

technique(s)

ELISA: suitable, immunocytochemistry: suitable, immunoprecipitation (IP): suitable, western blot: suitable

UniProt accession no.

Q16637

UniProt accession no.

Q16637

UniProt accession no.

Q16637

UniProt accession no.

O14893

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General description

The survival of motor neurons (SMN) protein is essential for the biogenesis of small nuclear RNA (snRNA)-ribonucleoproteins (snRNPs), the major components of the pre-mRNA splicing machinery. Though it is ubiquitously expressed, SMN deficiency causes the motor neuron degenerative disease spinal muscular atrophy (SMA). SMN deficiency has unexpected cell type-specific effects on the repertoire of snRNAs and mRNAs. It alters the stoichiometry of snRNAs and causes widespread pre-mRNA splicing defects in numerous transcripts of diverse genes, preferentially those containing a large number of introns, in SMN-deficient mouse tissues. The SMN complex plays a role in RNA metabolism and in splicing regulation.

Specificity

This antibody recognizes SMN.

Immunogen

Epitope: Unknown
His-tagged recombinant protein corresponding to human SMN.

Application

Anti-SMN Antibody, clone 2B1 is a Mouse Monoclonal Antibody for detection of SMN also known as Spinal muscular atrophy (Werdnig-Hoffmann disease Kugelberg-Welander disease) & has been validated in WB, ICC & IP.
Immunocytochemistry Analysis: 1:500 dilution from a representative lot detected SMN in HeLa cells.
Research Category
Epigenetics & Nuclear Function
Research Sub Category
RNA Metabolism & Binding Proteins

RNA Metabolism & Binding Proteins

Quality

Evaluated by Western Blot in HeLa cell lysate.

Western Blot Analysis: 0.5 µg/ml of this antibody detected SMN on 10 µg of HeLa cell lysate.

Target description

35 kDa was observed; however, the calculated molecular weight is 31.849 kDa

Physical form

Format: Purified
Protein G Purified
Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.05% sodium azide.

Storage and Stability

Stable for 1 year at 2-8°C from date of receipt.

Analysis Note

Control
HeLa cell lysate

Other Notes

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

Disclaimer

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.

Storage Class

12 - Non Combustible Liquids

wgk_germany

WGK 1

flash_point_f

Not applicable

flash_point_c

Not applicable


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Minoru Satoh et al.
Arthritis and rheumatism, 63(7), 1972-1978 (2011-03-23)
Autoantibodies in the systemic rheumatic diseases are clinically useful biomarkers of the diagnosis or of certain clinical characteristics. An unusual pattern of immunoprecipitation, in which the D, E, F, and G proteins of small nuclear RNPs (snRNP) but without other
Elena Hernandez-Gerez et al.
Annals of clinical and translational neurology, 7(9), 1580-1593 (2020-08-14)
The purpose of the study was to determine the extent and role of systemic hypoxia in the pathogenesis of spinal muscular atrophy (SMA). Hypoxia was assayed in vivo in early-symptomatic (postnatal day 5) SMA-model mice by pimonidazole and [18 F]-Fluoroazomycin
Lea Marie Esser et al.
International journal of molecular sciences, 24(21) (2023-11-14)
The survival motor neuron (SMN) complex is a multi-megadalton complex involved in post-transcriptional gene expression in eukaryotes via promotion of the biogenesis of uridine-rich small nuclear ribonucleoproteins (UsnRNPs). The functional center of the complex is formed from the SMN/Gemin2 subunit.
Lu Chen et al.
Nucleic acids research, 50(21), 12400-12424 (2022-08-11)
Trimethylguanosine synthase 1 (TGS1) is a highly conserved enzyme that converts the 5'-monomethylguanosine cap of small nuclear RNAs (snRNAs) to a trimethylguanosine cap. Here, we show that loss of TGS1 in Caenorhabditis elegans, Drosophila melanogaster and Danio rerio results in
Phosphorylation of pICln by the autophagy activating kinase ULK1 regulates snRNP biogenesis and splice activity of the cell.
Esser, et al.
Computational and structural biotechnology journal, 21, 2100-2109 (2023)

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