SMN2 monoclonal antibody (M01), clone 2B11-2A9
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More Files
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Specification
Product Description
Mouse monoclonal antibody raised against a full length recombinant SMN2.
Immunogen
SMN2 (AAH00908, 1 a.a. ~ 282 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
MAMSSGGSGGGVPEQEDSVLFRRGTGQSDDSDIWDDTALIKAYDKAVASFKHALKNGDICETSGKPKTTPKRKPAKKNKSQKKNTAASLQQWKVGDKCSAIWSEDGCIYPATIASIDFKRETCVVVYTGYGNREEQNLSDLLSPICEVANNIEQNAQENENESQVSTDESENSRSPGNKSDNIKPKSAPWNSFLPPPPPMPGPRLGPGKPGLKFNGPPPPPPPPPPHLLSCWLPPFPSGPPIIPPPPPICPDSLDDADALGSMLISWYMSGYHTGYYMEMLA
Host
Mouse
Reactivity
Human
Isotype
IgG2a kappa
Quality Control Testing
Antibody Reactive Against Recombinant Protein.
Western Blot detection against Immunogen (56.76 KDa) .
Storage Buffer
In 1x PBS, pH 7.4
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Tissue lysate)
SMN2 monoclonal antibody (M01), clone 2B11-2A9. Western Blot analysis of SMN2 expression in human colon.Western Blot (Cell lysate)
SMN2 monoclonal antibody (M01), clone 2B11-2A9 Western Blot analysis of SMN2 expression in IMR-32 ( Cat # L008V1 ).Western Blot (Recombinant protein)
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Immunoperoxidase of monoclonal antibody to SMN2 on formalin-fixed paraffin-embedded human heart tissue. [antibody concentration 1 ~ 10 ug/ml]Sandwich ELISA (Recombinant protein)
Detection limit for recombinant GST tagged SMN2 is approximately 0.3ng/ml as a capture antibody.ELISA
Immunofluorescence
Immunofluorescence of monoclonal antibody to SMN2 on HeLa cell. [antibody concentration 10 ug/ml] -
Gene Info — SMN2
Entrez GeneID
6607GeneBank Accession#
BC000908Protein Accession#
AAH00908Gene Name
SMN2
Gene Alias
BCD541, C-BCD541, FLJ76644, MGC20996, MGC5208, SMNC
Gene Description
survival of motor neuron 2, centromeric
Omim ID
601627Gene Ontology
HyperlinkGene Summary
This gene is part of a 500 kb inverted duplication on chromosome 5q13. This duplicated region contains at least four genes and repetitive elements which make it prone to rearrangements and deletions. The repetitiveness and complexity of the sequence have also caused difficulty in determining the organization of this genomic region. The telomeric and centromeric copies of this gene are nearly identical and encode the same protein. While mutations in the telomeric copy are associated with spinal muscular atrophy, mutations in this gene, the centromeric copy, do not lead to disease. This gene may be a modifier of disease caused by mutation in the telomeric copy. The critical sequence difference between the two genes is a single nucleotide in exon 7, which is thought to be an exon splice enhancer. Note that the nine exons of both the telomeric and centromeric copies are designated historically as exon 1, 2a, 2b, and 3-8. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. The full length protein encoded by this gene localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein. Four transcript variants encoding distinct isoforms have been described. [provided by RefSeq
Other Designations
OTTHUMP00000125236|OTTHUMP00000125237|gemin 1
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Interactome
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Disease
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Publication Reference
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Survival motor neuron (SMN) protein in the spinal anterior horn cells of patients with sporadic amyotrophic lateral sclerosis.
Yingshan Piao, Tomoyo Hashimoto, Sachiko Takahama, Akiyoshi Kakita, Takashi Komori, Takashi Morita, Hitoshi Takahashi, Toshio Mizutani, Kiyomitsu Oyanagi.
Brain Research 2011 Feb; 1372:152.
Application:IP, Hamster, CHO cells.
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The RNA binding protein hnRNP Q modulates the utilization of exon 7 in the survival motor neuron 2 (SMN2) gene.
Hung-Hsi Chen, Jan-Growth Chang, Ruei-Min Lu, Tsui-Yi Peng, Woan-Yuh Tarn.
Molecular and Cellular Biology 2008 Nov; 28(22):6929.
Application:WB-Ti, Mouse, Mouse brain, Mouse liver, Mouse testes.
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Survival motor neuron (SMN) protein in the spinal anterior horn cells of patients with sporadic amyotrophic lateral sclerosis.
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