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anti-SMN antibody :: Rabbit SMN Polyclonal Antibody

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Catalog # MBS7604263 (SPECIAL PROMOTIONAL PRICING for a limited time)
Unit / Price
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  0.1 mg  /  $290 +1 FREE 8GB USB
Product Name

SMN, Polyclonal Antibody

Popular Item
Full Product Name

SMN Rabbit Polyclonal

Product Synonym Names
C BCD541, Component of gems 1, FLJ76644, Gemin 1, SMN, SMN1,SMN1,SMN, SMN2, SMNC, SMNT, Survival motor neuron protein
Product Gene Name
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
OMIM
253300
3D Structure
ModBase 3D Structure for Q16637
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse, Rat
Specificity
Human, Mouse ,Rat; other species are not tested. Please decide the specificity by homology
Purity/Purification
Purification: Immunogen affinity purified
Purity: > = 95% as determined by SDS-PAGE
Form/Format
Liquid
Immunogen
Survival of motor neuron 2, centromeric
Buffer
PBS with 0.02% sodium azide and 50% glycerol pH 7.3
Preparation and Storage
-20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-SMN antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-SMN antibody
Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of anterior horn cells in the spinal cord and concomitant symmetrical muscle weakness and atrophy (PMID: 16364894 ). SMA is caused by deletion or mutations of the survival motor neuron (SMN1) gene. SMA patients lack a functional SMN1 gene, but they possess an intact SMN2 gene, which though nearly identical to SMN1, is only partially functional (PMID: 17355180). A large majority of SMN2 transcripts lack exon 7, resulting in production of a truncated, less stable SMN protein (PMID: 10369862). The level of SMN protein correlates with phenotypic severity of SMA. This antibody, 11708-1-AP, raised against the recombinant full-length human SMN2 protein, recognizes all isoforms of SMN protein.
Applications Tested/Suitable for anti-SMN antibody
ELISA, Immunohistochemistry (IHC), Immunofluorescence (IF), Western Blot (WB)
Application Notes for anti-SMN antibody
WB: 1:500-1:5000
IHC: 1:50-1:200
IF: 1:10-1:100

Immunohistochemistry of anti-SMN antibody
Immunohistochemistry of paraffin-embedded human brain using MBS7604263 (SMN2 antibody) at dilution of 1:100
anti-SMN antibody Immunohistochemistry (IHC) image
SDS-PAGE of anti-SMN antibody
HEK-293 cells were subjected to SDS PAGE followed by western blot with MBS7604263 (SMN2 antibody) at dilution of 1:1000
anti-SMN antibody SDS-PAGE image
NCBI/Uniprot data below describe general gene information for SMN. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
38kd
NCBI Official Full Name
survival motor neuron protein SMN
NCBI Official Synonym Full Names
survival of motor neuron 1, telomeric
NCBI Official Symbol
NCBI Official Synonym Symbols
SMA; SMN; SMA1; SMA2; SMA3; SMA4; SMA@; SMNT; BCD541; GEMIN1; TDRD16A; T-BCD541
  [Similar Products]
NCBI Protein Information
survival motor neuron protein
UniProt Protein Name
Survival motor neuron protein
UniProt Synonym Protein Names
Component of gems 1; Gemin-1
UniProt Gene Name
UniProt Synonym Gene Names
SMN; SMNT  [Similar Products]
UniProt Entry Name
SMN_HUMAN
NCBI Summary for SMN
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. Multiple transcript variants encoding distinct isoforms have been described. [provided by RefSeq, Jul 2014]
UniProt Comments for SMN
SMN: The SMN complex plays an essential role in spliceosomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing in the nucleus. It may also play a role in the metabolism of snoRNPs. Defects in SMN1 are the cause of spinal muscular atrophy autosomal recessive type 1 (SMA1). Spinal muscular atrophy refers to a group of neuromuscular disorders characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. Autosomal recessive forms are classified according to the age of onset, the maximum muscular activity achieved, and survivorship. The severity of the disease is mainly determined by the copy number of SMN2, a copy gene which predominantly produces exon 7-skipped transcripts and only low amount of full-length transcripts that encode for a protein identical to SMN1. Only about 4% of SMA patients bear one SMN1 copy with an intragenic mutation. SMA1 is a severe form, with onset before 6 months of age. SMA1 patients never achieve the ability to sit. Defects in SMN1 are the cause of spinal muscular atrophy autosomal recessive type 2 (SMA2). SMA2 is an autosomal recessive spinal muscular atrophy of intermediate severity, with onset between 6 and 18 months. Patients do not reach the motor milestone of standing, and survive into adulthood. Defects in SMN1 are the cause of spinal muscular atrophy autosomal recessive type 3 (SMA3). SMA3 is an autosomal recessive spinal muscular atrophy with onset after 18 months. SMA3 patients develop ability to stand and walk and survive into adulthood. Defects in SMN1 are the cause of spinal muscular atrophy autosomal recessive type 4 (SMA4). SMA4 is an autosomal recessive spinal muscular atrophy characterized by symmetric proximal muscle weakness with onset in adulthood and slow disease progression. SMA4 patients can stand and walk. Belongs to the SMN family. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: RNA processing; RNA-binding

Chromosomal Location of Human Ortholog: 5q13.2

Cellular Component: Cajal body; cytoplasm; cytosol; neuron projection; nucleoplasm; nucleus; perikaryon; SMN complex; Z disc

Molecular Function: identical protein binding; protein binding; RNA binding

Biological Process: nervous system development; nuclear import; spliceosomal snRNP biogenesis; spliceosome assembly; transcription termination

Disease: Spinal Muscular Atrophy, Type I; Spinal Muscular Atrophy, Type Ii; Spinal Muscular Atrophy, Type Iii; Spinal Muscular Atrophy, Type Iv
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Disclaimer
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