Anti-Survival Motor Neuron (SMN) Antibody
Our Anti-Survival Motor Neuron (SMN) mouse monoclonal primary antibody from PhosphoSolutions is prod
- 产品详情
- 实验流程
- 背景知识
Application ![]()
| WB, ICC |
---|---|
Primary Accession | Q16637 |
Host | Mouse |
Clonality | Monoclonal |
Isotype | IgG |
Clone Names | 4B7 |
Calculated MW | 31849 Da |
Gene ID | 6606;6607 |
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Other Names | 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 |
Target/Specificity | Survival Motor Neuron (SMN) protein, also known as Gemin1, is derived from the SMN gene which has two nearly identical copies located on chromosome 5q13, SMN1 and SMN2 (Lefebvre et al, 1995). SMA, Spinal Muscular Atrophy, is a neurodegenerative disease caused by mutations of the SMN gene that result in a loss of motor neurons and subsequent progressive limb and trunk muscular atrophy and paralysis (Crawford et al, 1996). SMN1 produces functional, full-length SMN protein, while SMN2 encodes a truncated form of SMN protein that is unstable and defective (Wolstencroft et al., 2005). SMN2 plays a key role in the development of SMA in that the number of SMN2 copies modulates disease severity (Monani et al, 2000). The SMN protein is expressed ubiquitously and found in the cytoplasm as well as nuclear Cajal bodies (Young et al, 2000). |
Dilution | WB~~1:1000 ICC~~N/A |
Format | Protein G Purified |
Storage | Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
Precautions | Anti-Survival Motor Neuron (SMN) Antibody is for research use only and not for use in diagnostic or therapeutic procedures. |
Shipping | Blue Ice |
For Research Use Only. Not For Use In Diagnostic Procedures.
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
Survival Motor Neuron (SMN) protein, also known as Gemin1, is derived from the SMN gene which has two nearly identical copies located on chromosome 5q13, SMN1 and SMN2 (Lefebvre et al, 1995). SMA, Spinal Muscular Atrophy, is a neurodegenerative disease caused by mutations of the SMN gene that result in a loss of motor neurons and subsequent progressive limb and trunk muscular atrophy and paralysis (Crawford et al, 1996). SMN1 produces functional, full-length SMN protein, while SMN2 encodes a truncated form of SMN protein that is unstable and defective (Wolstencroft et al., 2005). SMN2 plays a key role in the development of SMA in that the number of SMN2 copies modulates disease severity (Monani et al, 2000). The SMN protein is expressed ubiquitously and found in the cytoplasm as well as nuclear Cajal bodies (Young et al, 2000).

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