SMN1 Mouse Monoclonal Antibody [Clone ID: 4B7]

Specifications

Product Data
Clone Name	4B7
Applications	ICC, WB
Recommend Dilution	WB: 1:4000 ICC: 1:50-1:500
Reactivity	Human, Mouse, Rat
Host	Mouse
Clonality	Monoclonal
Immunogen	Recombinant human SMN protein.
Specificity	Specific for endogenous levels of the ~35 kDa survival motor neuron protein.
Formulation	10 mM HEPES (pH 7.5), 150 mM NaCl, 100 µg per ml BSA and 50% glycerol.
Concentration	lot specific
Purification	Protein G Purified
Conjugation	Unconjugated
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.
Predicted Protein Size	35
Gene Name	survival of motor neuron 1, telomeric
Database Link	Entrez Gene 6606 Human UniProt ID Q16637
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).
Synonyms	BCD541; Gemin-1; SMA; SMA1; SMA2; SMA3; SMA4; SMA@; SMN; SMNC; SMNT; T-BCD541
Note	Protein G purified tissue culture supernatant.
Reference Data

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