smn1 Antibody

Code CSB-PA895248XA01DIL
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Product Details

Full Product Name
Rabbit anti-Danio rerio (Zebrafish) (Brachydanio rerio) smn1 Polyclonal antibody
Uniprot No.
Target Names
Alternative Names
smn1 antibody; smn antibody; Survival motor neuron protein 1 antibody
Raised in
Rabbit
Species Reactivity
Danio rerio (Zebrafish) (Brachydanio rerio)
Immunogen
Recombinant Danio rerio (Zebrafish) (Brachydanio rerio) smn1 protein
Immunogen Species
Danio rerio (Zebrafish) (Brachydanio rerio)
Conjugate
Non-conjugated
Clonality
Polyclonal
Isotype
IgG
Purification Method
Antigen Affinity Purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA, WB (ensure identification of antigen)
Protocols
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Value-added Deliverables
① 200ug * antigen (positive control);
② 1ml * Pre-immune serum (negative control);
Quality Guarantee
① Antibody purity can be guaranteed above 90% by SDS-PAGE detection;
② ELISA titer can be guaranteed 1: 64,000;
③ WB validation with antigen can be guaranteed positive;
Lead Time
Made-to-order (14-16 weeks)
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Function
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. Required in motor neurons and proprioceptive neurons to ensure correct U12 intron splicing and proper levels of tmem41b mRNA. Required for the maturation of motor neuron axonal branches and dendrites.
Gene References into Functions
  1. we established that the additional supply of Smn protein in motoneurons is necessary for proper axonogenesis in a cell-autonomous manner PMID: 27273160
  2. In motor neurons, smn1 interacts with the RNA binding protein, HuD and regulates motoneuron development and function. PMID: 29061699
  3. our finding that elevation of Pgk1 levels or activity, via injection of Pgk1 mRNA or treatment with terazosin respectively, robustly ameliorated MN pathology in smn morphant zebrafish provides an initial demonstration that these pathways are amenable to therapeutic intervention. PMID: 28426667
  4. Reduction of Gemin2, unlike reduction of SMN, in zebrafish embryos does not directly cause motor axon outgrowth defects. PMID: 18000835
  5. SMN deficiency affects splicing and abundance of nrxn2a. This may explain the pre-synaptic defects at neuromuscular endplates in SMA pathophysiology. PMID: 24218366
  6. SMN is needed early in development of motoneuron dendrites and axons to develop normally and that this is essential for proper connectivity and movement. PMID: 23459934
  7. These results show that survival motor neuron functions in motor axon development and suggest that these early developmental defects may lead to subsequent motoneuron loss. PMID: 12952942
  8. Survival motor neuron has a novel function in motor neurons independent of small nuclear ribonucleoprotein (snRNP) biosynthesis. PMID: 17065443
  9. Since Gemin2 and SMN both function in snRNP biogenesis yet only SMN knockdown causes motor axon defects, these data are consistent with an additional role for SMN that is snRNP independent. PMID: 18000835
  10. Smn mutants in zebrafish survive to larval stages and exhibit presynaptic neuromuscular junction defects. PMID: 19592581

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Subcellular Location
Nucleus, gem. Nucleus, Cajal body. Cytoplasm. Cytoplasmic granule. Perikaryon. Cell projection, neuron projection. Cytoplasm, myofibril, sarcomere, Z line.
Protein Families
SMN family
Database Links
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301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)
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Address
7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA
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