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Product Name
SMN antibody
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Description
SMN Rabbit Polyclonal antibody. Positive IF detected in HepG2 cells. Positive WB detected in K-562 cells, HEK-293 cells, HepG2 cells, Jurkat cells. Observed molecular weight by Western-blot: 38 kDa
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Tested applications
ELISA, WB, IF
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Species reactivity
Human; other species not tested.
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Alternative names
C BCD541 antibody; Component of gems 1 antibody; FLJ76644 antibody; Gemin 1 antibody; SMN antibody; SMN1 antibody; SMN1 antibody; SMN antibody; SMN2 antibody; SMNC antibody; SMNT antibody; Survival motor neuron protein antibody
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Isotype
Rabbit IgG
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Preparation
This antibody was obtained by immunization of SMN recombinant protein (Accession Number: NM_022875). Purification method: Antigen Affinity purified.
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Clonality
Polyclonal
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Formulation
PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
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Storage instructions
Store at -20℃. DO NOT ALIQUOT
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Applications
Recommended Dilution:
WB: 1:1000-1:10000
IF: 1:20-1:200
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Validations
K-562 cells were subjected to SDS PAGE followed by western blot with Catalog No:115394(SMN2 antibody) at dilution of 1:2000
Immunofluorescent analysis of HepG2 cells using Catalog No:115394(SMN2 Antibody) at dilution of 1:50 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
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Background
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.
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