The survival of motor neurons(SMN) genes are the disease genes of spinal muscular atrophy(SMA), a common motor neuron degenerative disease. The level of SMN protein correlates with phenotypic severity of SMA. SMA patients lack a functional SMN1 gene, but they possess an intact SMN2 gene, which though nearly identical to SMN1, is only partially functional, because a large majority of SMN2 transcripts lack exon 7, resulting in production of a truncated, less stable SMN protein. This antibody can recognize human,mouse and rat SMN.
Categories
Primary Antibodies
Clonality
monoclonal
Host
Mouse
Immunogen
survival of motor neuron 2, centromeric
Isotype
IgG1
Molecular Weight
38kd
Reactivity
Human, Mouse, Rat
Regulatory
RUO
Synonyms
C BCD541, Component of gems 1, FLJ76644, Gemin 1, SMN, SMN1, SMN1,SMN, SMN2, SMNC, SMNT, Survival motor neuron protein
Uniprot
Q16637
Clone No
3D2
Research Area
Metabolism, Neuroscience
Form
liquid
Format
liquid
Purification
Protein A+G purification
Purity
>=95% as determined by SDS-PAGE
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20°C for 12 months(Avoid repeated freeze / thaw cycles.)