SMN1 抗体 (N-Term)
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北京 101111
Quick Overview for SMN1 抗体 (N-Term) (ABIN2778671)
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
- N-Term
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原理
- SMN1 Antibody - N-terminal region
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序列
- KAVASFKHAL KNGDICETSG KPKTTPKRKP AKKNKSQKKN TAASLQQWKV
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预测反应
- Dog: 100%, Guinea Pig: 85%, Horse: 93%, Human: 100%, Mouse: 100%, Pig: 93%, Rabbit: 85%, Rat: 92%
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产品特性
- This is a rabbit polyclonal antibody against SMN1. It was validated on Western Blot using a cell lysate as a positive control.
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纯化方法
- Affinity Purified
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免疫原
- The immunogen is a synthetic peptide directed towards the N terminal region of human SMN1
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应用备注
- Optimal working dilution should be determined by the investigator.
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说明
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We also sell a specific blocking peptide that can be used in combination with this antibody. You can find the blocking peptide under ABIN8113143
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限制
- 仅限研究用
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状态
- Liquid
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浓度
- 0.5 mg/mL
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缓冲液
- Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.
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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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注意事项
- prevent freeze-thaw cycles
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储存条件
- 4 °C,-20 °C
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储存方法
- For short term use, store at 2-8C up to 1 week. For long term storage, store at -20 °C in small aliquots to prevent freeze-thaw cycles.
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- SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))
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别名
- SMN1
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背景
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Background Information: SMN1 localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein.This gene is part of a 500 kb inverted duplication on chromosome 5q13. This duplicated region contains at least four genes and repetitive elements which make it prone to rearrangements and deletions. The repetitiveness and complexity of the sequence have also caused difficulty in determining the organization of this genomic region. The telomeric and centromeric copies of this gene are nearly identical and encode the same protein. However, mutations in this gene, the telomeric copy, are associated with spinal muscular atrophy, mutations in the centromeric copy do not lead to disease. The centromeric copy may be a modifier of disease caused by mutation in the telomeric copy. The critical sequence difference between the two genes is a single nucleotide in exon 7 which is thought to be an exon splice enhancer. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. The protein encoded by this gene localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein. Two transcript variants are produced by this gene.
Gene Name: Survival of motor neuron 1, telomeric
Alternative Symbols: SMA, SMN, SMA1, SMA2, SMA3, SMA4, SMA@, SMNT, BCD541, GEMIN1, TDRD16A, T-BCD541
Protein Name: Survival motor neuron protein
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分子量
- 28kDa
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基因ID
- 6606
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NCBI登录号
- NP_075012
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UniProt
- Q16637
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途径
- Ribonucleoprotein Complex Subunit Organization
抗原
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