bsm-70259M

DATASHEET

Host: Mouse

Target Protein: Survival Motor Neuron (SMN)

Specificity: Specific for endogenous levels of the ~35 kDa survival motor neuron protein.

Clonality: Monoclonal

Isotype: IgG

Entrez Gene: 6606

Swiss Prot: Q16637

Source: Recombinant human SMN protein.

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: 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.

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).

Size: 100ul

Concentration: Lot Dependent

Predicted Molecular Weight: 35

Cross Reactive Species: Human
Mouse
Rat

For research use only. Not intended for diagnostic or therapeutic use.