- 抗体类型:多克隆
- 抗体来源:兔
- 抗体应用:ELISA, IHC, IF, IP, WB
- 特异性:Human,Mouse,Rat; other species not tested.
产品详情
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产品名称
SMN antibody
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抗体类型
多克隆
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抗体来源
兔
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抗体亚型
兔IgG
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抗体描述
SMN Rabbit Polyclonal antibody. Positive IHC detected in human kidney tissue, human brain tissue, human heart tissue, human lung tissue, human ovary tissue, human placenta tissue, human skin tissue, human spleen tissue, human testis tissue. Positive IF detected in Hela cells, HepG2 cells. Positive WB detected in HEK-293 cells, A549 cells, HeLa cells, HepG2 cells, Jurkat cells, K-562 cells, mouse testis tissue. Positive IP detected in HEK-293. Observed molecular weight by Western-blot: 38 kDa; 66-70 kDa
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抗体应用
ELISA, IHC, IF, IP, WB
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应用推荐
Recommended Dilution:
WB: 1:500-1:5000
IP: 1:500-1:5000
IHC: 1:20-1:200
IF: 1:10-1:100
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特异性
Human,Mouse,Rat; other species not tested.
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蛋白别名
C BCD541, Component of gems 1, FLJ76644, Gemin 1, SMN, SMN1, SMN1, SMN, SMN2, SMNC, SMNT, Survival motor neuron protein
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制备方法
This antibody was obtained by immunization of SMN recombinant protein (Accession Number: NM_022875). Purification method: Antigen affinity purified.
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组分
PBS with 0.1% sodium azide and 50% glycerol pH 7.3.
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储存方法
Store at -20℃. DO NOT ALIQUOT
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背景介绍
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, 11708-1-AP, raised against the recombinant full-length human SMN2 protein, recognizes all isoforms of SMN protein.
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参考文献
- Kobayashi DT, Olson RJ, Sly L. Utility of survival motor neuron ELISA for spinal muscular atrophy clinical and preclinical analyses. PloS one. 6(8):e24269. 2011.
- Crawford TO, Paushkin SV, Kobayashi DT. Evaluation of SMN protein, transcript, and copy number in the biomarkers for spinal muscular atrophy (BforSMA) clinical study. PloS one. 7(4):e33572. 2012.
- Steinkellner H, Etzler J, Gmeiner BM, Laccone F. Detection of survival motor neuron protein in buccal cells through electrochemiluminescence-based assay. Assay and drug development technologies. 13(3):167-73. 2015.
- McGovern VL, Iyer CC, Arnold WD. SMN expression is required in motor neurons to rescue electrophysiological deficits in the SMNΔ7 mouse model of SMA. Human molecular genetics. 24(19):5524-41. 2015.
- Iyer CC, McGovern VL, Murray JD. Low levels of Survival Motor Neuron protein are sufficient for normal muscle function in the SMNΔ7 mouse model of SMA. Human molecular genetics. 24(21):6160-73. 2015.