Anti-Human/Mouse/Rat SMN1 Monoclonal Antibody

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 gene.
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Properties

Data Sheet Click for Datasheet
Catalog Number MA02132
Size 100μl
Host Type Mouse
Immunogen Fusion protein of SMN (Human,Mouse,Rat)
Specificity Expressed in a wide variety of tissues. Expressed at high levels in brain, kidney and liver, moderate levels in skeletal and cardiac muscle, and low levels in fibroblasts and lymphocytes. Also seen at high levels in spinal cord. Present in osteoclasts and mononuclear cells (at protein level).
Isotype IgG2b
Reacitivity Human,Mouse,Rat
Clone 600
Uniprot Q16637
Concentration None
Dilution WB 1:1500-1:6000
Formulation
Application WB,ELISA
Other Names BCD541,Component of gems 1,Gemin 1,Gemin-1,OTTHUMP00000125198,OTTHUMP00000223567,OTTHUMP00000223568,OTTHUMP00000224066,OTTHUMP00000226924,SMA 1,SMA 2,SMA 3,SMA 4,SMA,SMA@,SMA1,SMA2,SMA3,SMA4,SMN,SMN,SMN1,SMN2,SMNT,Survival motor neuron protein,Survival of motor neuron 1,telomeric,T-BCD541
Storage Lyophilized product: 5 years at 2 - 8°C; Solution: 2 years at -20°C.
Postscript For research use only, not for use in diagnostic procedures.

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