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IGHMBP2 抗体 (AA 271-355)

IGHMBP2 适用: 小鼠 WB, ELISA, IF (cc), IF (p), IHC (fro), IHC (p), ICC 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN1386574
发货至: 中国
  • 抗原 See all IGHMBP2 抗体
    IGHMBP2 (Immunoglobulin mu Binding Protein 2 (IGHMBP2))
    抗原表位
    • 14
    • 5
    • 3
    • 2
    • 1
    AA 271-355
    适用
    • 26
    • 20
    • 11
    • 2
    • 2
    • 2
    • 1
    • 1
    小鼠
    宿主
    • 34
    克隆类型
    • 34
    多克隆
    标记
    • 9
    • 3
    • 3
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This IGHMBP2 antibody is un-conjugated
    应用范围
    • 21
    • 12
    • 12
    • 9
    • 4
    • 2
    • 2
    • 1
    • 1
    Western Blotting (WB), ELISA, Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunocytochemistry (ICC)
    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
  • 应用备注
    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
  • 状态
    Liquid
    浓度
    1 μg/μL
    缓冲液
    0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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
    4 °C,-20 °C
    储存方法
    Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
    有效期
    12 months
  • 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

    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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