Learn about our comprehensive antibody validation methods to ensure monospecificity.  Antibody Validation>>

Survival of motor neuron 1, telomeric (SMN1 ) / Gemin 1 Antibody [SMN1/1596]

In Stock
Catalog Number Formulation Size Price
6606-MSM1-P0
Purified Ab with BSA and Azide at 200ug/ml
20ug
$229.00
6606-MSM1-P1
Purified Ab with BSA and Azide at 200ug/ml
100ug
$519.00
6606-MSM1-P1ABX
Purified Ab WITHOUT BSA and Azide at 1.0mg/ml
100ug
$519.00
Flat Rate Domestic: $60 | Orders outside the US - Contact Us for Order Information | Ships next business day

Applications & Dilutions

Applications Tested Dillution Protocol Note
Immunohistochemistry (IHC)
1-2ug/ml
30 min at RT. Staining of formalin-fixed tissues requires heating tissue sections in 10mM Tris with 1mM EDTA, pH 9.0, for 45 min at 95°C followed by cooling at RT for 20 minutes

Summary

Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations of SMN (survival of motor neuron) gene. SMN, also known as Gemin1, SMN1, SMNT and BCD541, exists as four isoforms produced by alternative splicing. SMN is oligomeric and forms a complex with Gemin2 (formerly SIP1), Gemin3 (a DEAD box RNA helicase), Gemin4, Gemin5 and Gemin6, as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in splicesomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. The SMN complex is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (gemini of the coiled bodies). Cytoplasmic SMN interacts with spliceosomal Sm proteins and facilitates their assembly onto U snRNAs, and nuclear SMN mediates recycling of pre-mRNA splicing factors. Nearly identical telomeric and centromeric forms of SMN encode the same protein; however, only mutations in the telomeric form are associated with the disease-state SMA. SMN is expresed in a wide variety of tissues including brain, kidney, liver, spinal cord and moderately in skeletal and cardiac muscle.

Product Properties & Targets

Antibody Type
Host
Mouse
Applications
Species Reactivity
Isotype / Light Chain
IgG1 / Kappa
Cellular Localization
Nucleus
Gene Name
Immunogen
Recombinant full-length human SMN1 protein
Alternate Names
survival motor neuron protein, BCD541 antibody; Component of gems 1 antibody; Gemin 1 antibody; Gemin-1 antibody; OTTHUMP00000125198 antibody; OTTHUMP00000223567 antibody; OTTHUMP00000223568 antibody; OTTHUMP00000224066 antibody; OTTHUMP00000226924 antibody; SMA 1 antibody; SMA 2 antibody; SMA 3 antibody; SMA 4 antibody; SMA antibody SMA@ antibody SMA1 antibody SMA2 antibody SMA3 antibody SMA4 antibody SMN antibody SMN_HUMAN antibody SMN1 antibody SMN2 antibody SMNT antibody Survival motor neuron protein antibody Survival of motor neuron 1, telomeric antibody T-BCD541 antibody, BTB and CNC homology 1; basic leucine zipper transcription factor 2

Database Links

Entrez Gene ID
SwissProt

Additional Information

Clone
SMN1/1596
Chromosome Location
5q13.2
Mol. Weight of Antigen
39kDa

Functions

  • 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.

Key References

  • Trifunov S, Natera-de Benito D, Carrera-García L, Codina A, Expósito-Escudero J, Ortez C, Medina J, Torres Alcala S, Bernal S, Alias L, Badosa C, Balsells S, Alcolea D, Nascimento A, Jimenez-Mallebrera C. Full-Length SMN Transcript in Extracellular Vesicles as Biomarker in Individuals with Spinal Muscular Atrophy Type 2 Treated with Nusinersen. J Neuromuscul Dis. 2023;10(4):653-665. doi: 10.3233/JND-230012. PMID: 37038823; PMCID: PMC10357204.

Storage & Stability

Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C. Antibody is stable for 24 months. Non-hazardous. No MSDS required.

Limitations

This antibody is available for research use only and is not approved for use in diagnosis.

Supplied as

200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/ml.

Warranty

There are no warranties, expressed or implied, which extend beyond this description. Company is not liable for any personal injury or economic loss resulting from this product.

Reviews

There are no reviews yet.

Be the first to review “Survival of motor neuron 1, telomeric (SMN1 ) / Gemin 1 Antibody”

Your email address will not be published. Required fields are marked *

PARTNERSHIP OPPORTUNITIES

We hold Exclusive rights to 10,000 recombinant and hybridoma antibody products, available for Licensing or Collaboration.

LETS TALK