Anti-SMN-Exon7 antibody

Product Name Anti-SMN-Exon7 antibody

Catalog No PAab08036

Packing 100ug

Form liquid

Alternative Name

Purification Protein A+G purification

Purity 95% as determined by SDS-PAGE

Host Mouse

Isotype IgG1

Storage PBS with 0.02% sodium azide and 50% glycerol pH 7.3 , -20Centigrade for 24 months (Avoid repeated freeze / thaw cycles.)

Background/ FUNCTION

Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of anterior horn cells in the spinal cord and concomitant symmetrical muscle weakness and atrophy (PMID: 16364894 ). SMA is caused by deletion or mutations of the survival motor neuron (SMN1) gene. SMA patients lack a functional SMN1 gene, but they possess an intact SMN2 gene, which though nearly identical to SMN1, is only partially functional (PMID: 17355180). A large majority of SMN2 transcripts lack exon 7, resulting in production of a truncated, less stable SMN protein (PMID: 10369862). The level of SMN protein correlates with phenotypic severity of SMA. This antibody, 60255-1-Ig, raised against the C-ternimal region (275-294aa) encoded by the exon 7.

Immunogen survival of motor neuron 1, telomeric

Specificity Human,Mouse

Tested Application IF,WB

Recommended dilution

IF use

Immunofluorescent analysis of HepG2 cells using Anti-SMN-Exon7 antibody at dilution of 1:50 and Alexa Fluor 488-conjugated AffiniPure Goat Anti-Mouse IgG(H+L)

WB use

HEK-293 cells were subjected to SDS PAGE followed by western blot with Anti-SMN-Exon7 antibody at dilution of 1:1000 incubated at room temperature for 1.5 hours

Protein Information

Gene ID6606

Uniprot ID

Calculated MW  32 kDa