SMN1 抗体 (AA 31-100)
Quick Overview for SMN1 抗体 (AA 31-100) (ABIN1387722)
抗原
See all SMN1 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 31-100
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交叉反应
- 小鼠
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预测反应
- Human,Rat,Dog,Cow,Pig,Rabbit
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纯化方法
- Purified by Protein A.
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免疫原
- KLH conjugated synthetic peptide derived from human Gemin 1
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亚型
- IgG
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应用备注
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WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500 -
限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1 μg/μL
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缓冲液
- 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
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储存液
- ProClin
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注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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储存条件
- 4 °C,-20 °C
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储存方法
- Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
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有效期
- 12 months
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- SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))
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别名
- Gemin 1/SMA
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背景
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Synonyms: Component of gems 1, Gemin 1, Gemin-1, Gemin1, SMA 1, SMA 2, SMA 3, SMA 4, SMA, SMA1, SMA2, SMA3, SMA4, SMN 1, SMN, SMN-1, SMN_HUMAN, SMN1, SMN2, SMNT, Survival motor neuron protein, Survival of motor neuron 1 telomeric, survival of motor neuron 1, Survival of motor neuron 1, telomeric, T-BCD541, BCD541, SMN_HUMAN.
Background: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations of SMN (survival of motor neuron) gene. SMN, also known as Gemin1, SMN1, SMNT and BCD541, exists as four isoforms produced by alternative splicing. SMN is oligomeric and forms a complex with Gemin2 (formerly SIP1), Gemin3 (a DEAD box RNA helicase), Gemin4, Gemin5 and Gemin6, as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in splicesomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. The SMN complex is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (gemini of the coiled bodies). Cytoplasmic SMN interacts with spliceosomal Sm proteins and facilitates their assembly onto U snRNAs, and nuclear SMN mediates recycling of pre-mRNA splicing factors. Nearly identical telomeric and centromeric forms of SMN encode the same protein, however, only mutations in the telomeric form are associated with the disease-state SMA. SMN is expresed in a wide variety of tissues including brain, kidney, liver, spinal cord and moderately in skeletal and cardiac muscle.
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途径
- Ribonucleoprotein Complex Subunit Organization
抗原
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