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Alternative name
C BCD541, Component of gems 1, FLJ76644, Gemin 1, SMN, SMN1, SMN1,SMN, SMN2, SMNC, SMNT, Survival motor neuron protein antibody
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Catalog
A008030
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Size100μg
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Formliquid
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Purity≥95% as determined by SDS-PAGE
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StoragePBS with 0.02% sodium azide and 50% glycerol pH 7.3 , -20℃ for 24 months (Avoid repeated freeze / thaw cycles.)
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ClonalityPolyclonal Antibody
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Host Rabbit
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Species ReactivityHuman,Mouse ,Rat
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Applications Tested/SuitableELISA,IHC,IF,IP,WB
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PurificationImmunogen affinity purified
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Immunogensurvival of motor neuron 2, centromeric
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IHCImmunohistochemistry of paraffin-embedded human brain using A008030(SMN2 antibody) at dilution of 1:100
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Western BlotHEK-293 cells were subjected to SDS PAGE followed by western blot with A008030(SMN2 antibody) at dilution of 1:1000
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Recommended dilutionWB : 1:500-1:5000
IP : 1:500-1:5000
IHC : 1:50-1:200
IF : 1:10-1:100
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Product Description specificalSpinal 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, 11708-1-AP, raised against the recombinant full-length human SMN2 protein, recognizes all isoforms of SMN protein.
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Gene nameSMN2
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Uniprot IDQ16637
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Calculated M.W.38 kDa
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Observed M.W.38 kDa
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