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Survival Motor Neuron (SMN) Antibody

Applications

  • WB
  • ICC

Reactivity

  • Human
  • Mouse
  • Rat
Overview
Catalog # bsm-70259M
Product Name Survival Motor Neuron (SMN) Antibody
Applications WB, ICC
Specificity Specific for endogenous levels of the ~35 kDa survival motor neuron protein.
Reactivity Human, Mouse, Rat
Specifications
Conjugation Unconjugated
Host Mouse
Source Recombinant human SMN protein.
Clonality Monoclonal
Clone # 4B7
Isotype IgG
Concentration Lot Dependent
Purification Purified by Protein G.
Storage Buffer 10 mM HEPES (pH 7.5), 150 mM NaCl, 100 µg per ml BSA and 50% glycerol.
Storage Condition Storage at -20°C is recommended, as aliquots may be taken without freeze/thawing due to presence of 50% glycerol. Stable for at least 1 year at -20°C.
Target
Gene ID 6606
Swiss Prot Q16637
Synonyms 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
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).
Application Dilution
WB 1:300-5000
ICC 1:100-500