SMN1/SMN2 polyclonal antibody
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Specification
Product Description
Goat polyclonal antibody raised against synthetic peptide of SMN1/SMN2.
Immunogen
A synthetic peptide corresponding to human SMN1, SMN2.
Sequence
C-DESENSRSPGNKSDN
Host
Goat
Theoretical MW (kDa)
28.5, 31.8, 30.5, 28
Reactivity
Human
Specificity
This antibody is expected to recognize isoforms b and d of SMN1 (NP_075012.1 and NP_000335.1) and all reported isoforms of SMN2 (NP_075013.1, NP_075014.1, NP_075015.1 and NP_059107.1).
Form
Liquid
Purification
Antigen affinity purification
Concentration
0.5 mg/mL
Quality Control Testing
Antibody Reactive Against Synthetic Peptide.
Recommend Usage
ELISA (1:1000)
Western Blot (2-4 ug/mL)
The optimal working dilution should be determined by the end user.Storage Buffer
In Tris saline, pH 7.3 (0.5% BSA, 0.02% sodium azide)
Storage Instruction
Store at -20°C.
Aliquot to avoid repeated freezing and thawing.Note
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Applications
Western Blot (Tissue lysate)
The SMN1/SMN2 polyclonal antibody (Cat # PAB7236) (2 ug/mL) staining of human brain (cerebellum) lysate (35 ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.Enzyme-linked Immunoabsorbent Assay
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Gene Info — SMN1
Entrez GeneID
6606Gene Name
SMN1
Gene Alias
BCD541, SMA, SMA1, SMA2, SMA3, SMA4, SMA@, SMN, SMNT, T-BCD541
Gene Description
survival of motor neuron 1, telomeric
Gene 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. However, mutations in this gene, the telomeric copy, are associated with spinal muscular atrophy; mutations in the centromeric copy do not lead to disease. The centromeric copy 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 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. Two transcript variants encoding distinct isoforms have been described. [provided by RefSeq
Other Designations
OTTHUMP00000125198|gemin 1
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Gene Info — SMN2
Entrez GeneID
6607Gene 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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Axonal-SMN (a-SMN), a protein isoform of the survival motor neuron gene, is specifically involved in axonogenesis.
Setola V, Terao M, Locatelli D, Bassanini S, Garattini E, Battaglia G.
PNAS 2007 Feb; 104(6):1959.
Application:IF, WB, Human, Rat, HeLa, NSC34 cells, Rat spinal cord.
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Axonal-SMN (a-SMN), a protein isoform of the survival motor neuron gene, is specifically involved in axonogenesis.
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