SMN1 / SMN2 (AA 22-52), (N-Term) 抗体
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- 抗原
- SMN1 / SMN2
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抗原表位
- AA 22-52, N-Term
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适用
- 人
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宿主
- 小鼠
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克隆类型
- 单克隆
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标记
- 非结合性
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应用范围
- Western Blotting (WB)
- 品牌
- Picoband™
- 序列
- RRGTGQSDDS DIWDDTALIK AYDKAVASFK H
- 交叉反应 (详细)
- No cross reactivity with other proteins.
- 产品特性
- Mouse IgG monoclonal antibody for SMN1/2 detection. Tested with WB in Human.
- 免疫原
- A synthetic peptide corresponding to a sequence at the N-terminus of human SMN1/2(22-52aa RRGTGQSDDSDIWDDTALIKAYDKAVASFKH), identical to the related mouse and rat sequences.
- 克隆位点
- 2B10
- 亚型
- IgG1
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- 应用备注
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Recommended Detection Systems: Enhanced Chemiluminescent Kit with anti-Rabbit IgG (ABIN921124) for Western blot.
Application Details: Western blot, 0.1-0.5 μg/mL
- 限制
- 仅限研究用
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- 状态
- Lyophilized
- 溶解方式
- Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
- 缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg Sodium azide.
- 储存液
- Sodium azide
- 注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
- 储存条件
- 4 °C,-20 °C
- 储存方法
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At -20°C for one year. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for a longer time. Avoid repeated freezing and thawing.
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- 抗原
- SMN1 / SMN2
- 别名
- SMN1/2
- 背景
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Synonyms: Survival motor neuron protein, Component of gems 1, Gemin-1, SMN1, SMN, SMNT, SMN2, SMNC
Tissue Specificity: Expressed in a wide variety of tissues. Expressed at high levels in brain, kidney and liver, moderate levels in skeletal and cardiac muscle, and low levels in fibroblasts and lymphocytes. Also seen at high levels in spinal cord. Present in osteoclasts and mononuclear cells (at protein level).
Background: 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. Note that the nine exons of both the telomeric and centromeric copies are designated historically as exon 1, 2a, 2b, and 3-8. 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. Multiple transcript variants encoding distinct isoforms have been described.
- UniProt
- Q16637
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