IGHMBP2 抗体 (AA 271-355) (Biotin)
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- 抗原 See all IGHMBP2 抗体
- IGHMBP2 (Immunoglobulin mu Binding Protein 2 (IGHMBP2))
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抗原表位
- AA 271-355
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适用
- 小鼠
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This IGHMBP2 antibody is conjugated to Biotin
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应用范围
- Western Blotting (WB), ELISA, Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
- 交叉反应
- 小鼠
- 预测反应
- Human,Rat,Cow,Sheep,Pig
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human SMUBP2
- 亚型
- IgG
- Top Product
- Discover our top product IGHMBP2 Primary Antibody
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- 应用备注
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WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500 - 限制
- 仅限研究用
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- 状态
- Liquid
- 浓度
- 1 μg/μL
- 缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- 储存液
- ProClin
- 注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- 储存条件
- -20 °C
- 储存方法
- Store at -20°C for 12 months.
- 有效期
- 12 months
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- 抗原
- IGHMBP2 (Immunoglobulin mu Binding Protein 2 (IGHMBP2))
- 别名
- SMUBP2 (IGHMBP2 产品)
- 别名
- NV12364 antibody, CATF1 antibody, HCSA antibody, HMN6 antibody, SMARD1 antibody, SMUBP2 antibody, ZFAND7 antibody, AEP antibody, Catf1 antibody, RIPE3b1 antibody, Smbp-2 antibody, Smbp2 antibody, Smubp2 antibody, nmd antibody, sma antibody, RIP-1 antibody, RIP1 antibody, immunoglobulin mu binding protein 2 antibody, IGHMBP2 antibody, Ighmbp2 antibody, ighmbp2 antibody
- 背景
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Synonyms: AEP, Antreeze enhancer binding protein, ATP-dependent helicase IGHMBP2, Cardiac transcription factor 1, Cardiac transcription factor1, CATF 1, CATF1, DNA-binding protein SMUBP-2, GF-1, Glial factor 1, HCSA, HMN 6, HMN6, IGHMBP 2, Ighmbp2, Immunoglobulin mu binding protein 2, Immunoglobulin mu binding protein2, Immunoglobulin mu-binding protein 2, Immunoglobulin S mu binding protein 2, Immunoglobulin S mu binding protein2, RIPE3 b1, RIPE3b 1, RIPE3b1, SMARD 1, SMARD1, SMBP2_HUMAN, SMUBP 2.
Background: IGHMBP2 is a 993 amino acid nuclear and cytoplasmic protein that is ubiquitously expressed. Belonging to the DNA2/NAM7 helicase family, IGHMBP2 is a 5' to 3' helicase that unwinds RNA and DNA duplexes in an ATP-dependent reaction. IGHMBP2 also acts as a transcriptional regulator and is necessary for transcriptional activation of the flounder liver-type antifreeze protein gene. IGHMBP2 exists as a homooligomer and is part of the cytosolic ribonucleoprotein complex. Mutations in the gene encoding IGHMBP2 are suggested to lead to distal hereditary motor neuronopathy type 6 (HMN6), also known as spinal muscular atrophy distal autosomal recessive 1 (DSMA1) or spinal muscular atrophy with respiratory distress 1 (SMARD1). HMN6 is characterized by weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs and severe respiratory distress.
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