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Survival of Motor Neuron 1, Telomeric Proteins (SMN1)

SMN1 is part of a 500 kb inverted duplication on chromosome 5q13. Additionally we are shipping SMN1 Antibodies (104) and SMN1 Kits (31) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
SMN1 20589  
SMN1 6606 Q16637
SMN1 64301 O35876
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Top SMN1 Proteins at antibodies-online.com

Showing 6 out of 8 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Wheat germ Human GST tag 10 μg Log in to see 9 Days
$405.71
Details
Yeast Cow His tag   1 mg Log in to see 56 to 66 Days
$2,755.50
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Yeast Dog His tag   1 mg Log in to see 56 to 66 Days
$2,755.50
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Yeast Rat His tag   1 mg Log in to see 56 to 66 Days
$2,762.83
Details
Yeast Macaca fascicularis His tag   1 mg Log in to see 56 to 66 Days
$2,775.67
Details

SMN1 Proteins by Origin and Source

Origin Expressed in Conjugate
Mouse (Murine)

Human

Rat (Rattus)

More Proteins for Survival of Motor Neuron 1, Telomeric (SMN1) Interaction Partners

Cow (Bovine) Survival of Motor Neuron 1, Telomeric (SMN1) interaction partners

  1. Data show that the coding sequence of survival of motor neuron 2 (SMN2) differs from that of survival motor neuron 1 (SMN1) by a single nucleotide (c.840C>T) at codon 280 in exon 7.

  2. SMN (show SNRPN Proteins) protein functions in cytoplasmic Sm-core assembly and in the recruitment of the snRNA cap hypermethylase

Mouse (Murine) Survival of Motor Neuron 1, Telomeric (SMN1) interaction partners

  1. muscle does not appear to require high levels of SMN (show STMN1 Proteins) above what is produced by two copies of SMN2

  2. Findings demonstrate that high expression of SMN (show STMN1 Proteins) in the motor neuron is both necessary and sufficient for proper function of the motor unit. In addition, SMN (show STMN1 Proteins) high expression in neurons and glia has a major impact on survival.

  3. This study identifies pathways related to the function of Smn (show STMN1 Proteins) and associated with differential motor unit vulnerability, thus presenting a number of exciting targets for future therapeutic development.

  4. Smn (show STMN1 Proteins) complex deficiency caused constipation, delayed gastric emptying, slow intestinal transit and reduced colonic motility.

  5. Primary cell culture and two different SMA model mice to demonstrate that reduced levels of Smn (show STMN1 Proteins) lead to a profound disruption in the expression of myogenic genes.

  6. Results suggest that SMN (show STMN1 Proteins) plays a role in the maintenance of pluripotent embryonic stem cells and neuronal differentiation in mice.

  7. AAV9-mediated SMN (show STMN1 Proteins) gene therapy elicits cure for spinal muscular atrophy.

  8. Data show that changes in U12 introns-dependent splicing become apparent after prolonged/extensive survival motor neuron proteins SMN (show STMN1 Proteins) depletion.

  9. This work both reveals a new autoregulatory pathway governing SMN (show STMN1 Proteins) expression, and identifies a new mechanism through which SMN (show STMN1 Proteins) can modulate specific mRNA expression via Gemin5 (show GEMIN5 Proteins).

  10. SMN (show STMN1 Proteins) is involved in the axonal translocation of hnRNP R (show HNRNPR Proteins) and hnRNP R (show HNRNPR Proteins)-bound RNA/protein complexes.

Human Survival of Motor Neuron 1, Telomeric (SMN1) interaction partners

  1. The results of this study show that, the plasmid containing UTR elements causes to twice more SMN (show STMN1 Proteins) gene expression in transfected cells.

  2. Data show that the coding sequence of survival of motor neuron 2 (SMN2) differs from that of survival motor neuron 1 (SMN1) by a single nucleotide (c.840C>T) at codon 280 in exon 7.

  3. The Cajal bodies fail to recruit SMN (show STMN1 Proteins) and spliceosomal snRNPs, but contain the proteasome activator PA28, a molecular marker associated with the cellular stress response.

  4. PLS3 (show PLS3 Proteins) is a genuine spinal muscular atrophy protective modifier in SMN1-deleted individuals

  5. Measurements of SMN (show STMN1 Proteins) and PLS3 (show PLS3 Proteins) transcript and protein levels in induced pluripotent stem cell-derived motor neurons show limited value as Spinal muscular atrophy biomarkers.

  6. The diverse a-SMN (show STMN1 Proteins) vs FL-SMN (show STMN1 Proteins) C-terminus may dictate different protein interactions and complex formation explaining the different localization and role in the neuronal compartment, and the lower expression and stability of a-SMN (show STMN1 Proteins).

  7. SMN and symmetric arginine dimethylation of RNA polymerase II C-terminal domain control transcriptional termination

  8. Among 43 identified patients with spinal muscular atrophy, 37 (86.0%) showed homozygous deletion of SMN1 exon 7.

  9. Study detected 3 small mutations in 4 patients without homozygous deletion of the SMN1 gene, suggested that about 4% of spinal muscular atrophy patients have subtle mutations and might be considered in laboratory examination

  10. SMN1 Gene Point Mutations in Type I-IV Proximal Spinal Muscular Atrophy Patients with a Single Copy of SMN1

Pig (Porcine) Survival of Motor Neuron 1, Telomeric (SMN1) interaction partners

  1. first cloning and identification of the swine SMN1 gene and show that there is significant sequence homology between swine and human SMN (show SNRPN Proteins) throughout the coding region

SMN1 Protein Profile

Protein 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.

Gene names and symbols associated with SMN1

  • survival of motor neuron 1, telomeric (SMN1)
  • immunoglobulin mu binding protein 2 (Ighmbp2)
  • survival motor neuron 1 (Smn1)
  • survival of motor neuron 1, telomeric (Smn1)
  • survival of motor neuron 1, telomeric-like (LOC100348318)
  • AEP protein
  • AI849087 protein
  • BCD541 protein
  • Catf1 protein
  • GEMIN1 protein
  • nmd protein
  • RIPE3b1 protein
  • sma protein
  • SMA1 protein
  • SMA2 protein
  • SMA3 protein
  • SMA4 protein
  • SMA@ protein
  • Smbp-2 protein
  • Smbp2 protein
  • Smn protein
  • SMN2 protein
  • SMNT protein
  • Smubp2 protein
  • T-BCD541 protein
  • TDRD16A protein

Protein level used designations for SMN1

survival of motor neuron 2, centromeric , survival motor neuron protein , ATP-dependent helicase IGHMBP2 , DNA-binding protein SMUBP-2 , antifreeze enhancer-binding protein , cardiac transcription factor 1 , immunoglobulin S mu binding protein 2 , immunoglobulin mu-binding protein 2 , neuromuscular degeneration , p110 subunit , survival of motor neuron protein , component of gems 1 , gemin-1 , survival motor neuron 1 protein , tudor domain containing 16A , survival motor neuron 1

GENE ID SPECIES
461829 Pan troglodytes
677703 Macaca mulatta
100171813 Pongo abelii
100308165 Bos taurus
20589 Mus musculus
20595 Mus musculus
6606 Homo sapiens
100170853 Sus scrofa
64301 Rattus norvegicus
100713418 Cavia porcellus
281492 Bos taurus
101110178 Ovis aries
100348318 Oryctolagus cuniculus
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