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anti-SMN antibody :: Rabbit SMN Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS7604263 (SPECIAL PROMOTIONAL PRICING for a limited time)
Unit / Price
0.1 mg  /  $180 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

SMN, Polyclonal Antibody

★Popular Item★
 Also Known As   

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   

anti-SMN antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    253300
 3D Structure    ModBase 3D Structure for Q16637
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Mouse, Rat
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 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
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 Immunogen    Survival of motor neuron 2, centromeric
 Buffer    PBS with 0.02% sodium azide and 50% glycerol pH 7.3
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 Preparation and Storage    -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)
 ISO Certification    Manufactured in an ISO 9001:2008 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.
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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
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 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
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 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
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NCBI/Uniprot data below describe general gene information for SMN. It may not necessarily be applicable to this product.
 NCBI GI #    1737214
 NCBI GeneID    6606
 NCBI Accession #    AAC52048.1 [Other Products]
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 UniProt Primary Accession #    Q16637 [Other Products]
 UniProt Secondary Accession #    Q13119; Q549U5; Q96J51; A8K0V4 [Other Products]
 UniProt Related Accession #    Q16637 [Other Products]
 Molecular Weight    38kd
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 NCBI Official Full Name    survival motor neuron protein SMN
 NCBI Official Synonym Full Names    survival of motor neuron 1, telomeric
 NCBI Official Symbol    SMN1 [Similar Products]
 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    SMN1 [Similar Products]
 UniProt Synonym Gene Names    SMN; SMNT [Similar Products]
 UniProt Entry Name    SMN_HUMAN
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 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]
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 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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 Research Articles on SMN    1. Measurements of SMN and PLS3 transcript and protein levels in induced pluripotent stem cell-derived motor neurons show limited value as Spinal muscular atrophy biomarkers.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-SMN antibodyPathways associated with anti-SMN antibody
 Reference Product  PubMed Publications
 SMN2 antibody  >520 publications with SMN and SMN2
 GEMIN2 antibody  >14 publications with SMN and GEMIN2
 STRAP antibody  >1 publications with SMN and STRAP
 GEMIN7 antibody  >1 publications with SMN and GEMIN7
 GEMIN4 antibody  >1 publications with SMN and GEMIN4
 SNRPB antibody  >1 publications with SMN and SNRPB
 DDX20 antibody  >1 publications with SMN and DDX20
 Products by Pathway  Pathway Diagram
 Gene Expression Pathway antibodies  Gene Expression Pathway Diagram
 Metabolism Of Non-coding RNA Pathway antibodies  Metabolism Of Non-coding RNA Pathway Diagram
 RNA Transport Pathway antibodies  RNA Transport Pathway Diagram
 RNA Transport Pathway antibodies  RNA Transport Pathway Diagram
 Survival Motor Neuron (SMN) Complex Pathway antibodies  Survival Motor Neuron (SMN) Complex Pathway Diagram
 Survival Motor Neuron (SMN) Complex Pathway antibodies  Survival Motor Neuron (SMN) Complex Pathway Diagram
 SnRNP Assembly Pathway antibodies  SnRNP Assembly Pathway Diagram
Diseases associated with anti-SMN antibodyOrgans/Tissues associated with anti-SMN antibody
 Disease Name  Pubmed Publications
 Atrophy Antibodies  >873 publications with SMN and Atrophy
 Muscular Atrophy, Spinal Antibodies  >872 publications with SMN and Muscular Atrophy, Spinal
 Disease Models, Animal Antibodies  >153 publications with SMN and Disease Models, Animal
 Spinal Muscular Atrophies of Childhood Antibodies  >134 publications with SMN and Spinal Muscular Atrophies of Childhood
 Death Antibodies  >125 publications with SMN and Death
 Nerve Degeneration Antibodies  >60 publications with SMN and Nerve Degeneration
 Weight Loss Antibodies  >24 publications with SMN and Weight Loss
 Fibrosis Antibodies  >11 publications with SMN and Fibrosis
 Embryo Loss Antibodies  >9 publications with SMN and Embryo Loss
 Cardiovascular Diseases Antibodies  >8 publications with SMN and Cardiovascular Diseases
 Organ/Tissue Name  Pubmed Publications
 Nerve Antibodies  >337 publications with SMN and Nerve
 Muscle Antibodies  >238 publications with SMN and Muscle
 Brain Antibodies  >84 publications with SMN and Brain
 Blood Antibodies  >73 publications with SMN and Blood
 Embryonic Tissue Antibodies  >33 publications with SMN and Embryonic Tissue
 Heart Antibodies  >16 publications with SMN and Heart
 Skin Antibodies  >15 publications with SMN and Skin
 Bone Antibodies  >11 publications with SMN and Bone
 Vascular Antibodies  >9 publications with SMN and Vascular
 Liver Antibodies  >9 publications with SMN and Liver
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