SMN1 抗体
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北京 101111
Quick Overview for SMN1 抗体 (ABIN7865272)
抗原
See all SMN1 抗体适用
宿主
克隆类型
标记
应用范围
克隆位点
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原理
- Survival of motor neuron 1, telomeric (SMN1 ) / Gemin 1 Antibody
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特异性
- 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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纯化方法
- 200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G.
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免疫原
- Recombinant full-length human SMN1 protein
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亚型
- IgG1, kappa
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anti-Survival of Motor Neuron 1, Telomeric (SMN1) antibody
SMN1 适用: 人, 猴 WB, ELISA, IHC, ICC 宿主: 小鼠 Monoclonal 5H1 unconjugated
anti-Survival of Motor Neuron 1, Telomeric (SMN1) antibodySMN1 适用: 人, 猴 WB, ELISA, IHC, FACS 宿主: 小鼠 Monoclonal 2F1 unconjugated
anti-Survival of Motor Neuron 1, Telomeric (SMN1) (AA 22-52), (N-Term) antibodySMN1 适用: 人, 小鼠, 大鼠 WB, IHC, IF, ICC, FACS 宿主: 兔 Polyclonal unconjugated
anti-Survival of Motor Neuron 1, Telomeric (SMN1) (AA 80-150) antibodySMN1 适用: 人 WB, IHC 宿主: 兔 Polyclonal unconjugated
anti-Survival of Motor Neuron 1, Telomeric (SMN1) (N-Term) antibodySMN1 适用: 人, 小鼠, 大鼠, 犬, Pig, 兔, 豚鼠, 马 WB 宿主: 兔 Polyclonal unconjugated
anti-Survival of Motor Neuron 1, Telomeric (SMN1) (AA 2-294) antibodySMN1 适用: 人 WB, IF, ICC 宿主: 兔 Polyclonal unconjugated
anti-Survival of Motor Neuron 1, Telomeric (SMN1) (AA 1-140) antibodySMN1 适用: 人 WB, IF 宿主: 小鼠 Polyclonal unconjugated
anti-Survival of Motor Neuron 1, Telomeric (SMN1) (AA 3-194) antibodySMN1 适用: 人 WB, ELISA, IHC, IF 宿主: 兔 Polyclonal unconjugated
anti-Survival of Motor Neuron 1, Telomeric (SMN1) antibodySMN1 适用: 人 WB, IHC 宿主: 兔 Monoclonal unconjugated
anti-Survival of Motor Neuron 1, Telomeric (SMN1) antibodyKO Validated SMN1 适用: 人 WB, FACS 宿主: 兔 Monoclonal 25GB1840 unconjugated Recombinant Antibody
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应用备注
- Optimal working dilution should be determined by the investigator.
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限制
- 仅限研究用
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浓度
- 200 μg/mL
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缓冲液
- Prepared in 10 mM PBS with 0.05 % BSA & 0.05 % azide.
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- 4 °C
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储存方法
- Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C. Antibody is stable for 24 months. Non-hazardous. No MSDS required.
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有效期
- 24 months
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- SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))
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别名
- Survival of motor neuron 1, telomeric
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背景
- BTB and CNC homology 1, basic leucine zipper transcription factor 2
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分子量
- 39kDa
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基因ID
- 6606, 202179
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UniProt
- Q16637
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途径
- Ribonucleoprotein Complex Subunit Organization
抗原
-
