HIBADH Rabbit pAb(bs-15483R)-50ul/100ul/200ul

HIBADH Rabbit pAb(bs-15483R)-5

0ul/100ul/200ul
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  • ¥1180 - 2800
  • Bioss已认证
  • bs-15483R
  • 2025年10月24日
  • 产品信息以Bioss网站为准
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      50ul/100ul/200ul

    规格:50ul产品价格:¥1180.0
    规格:100ul产品价格:¥1980.0
    规格:200ul产品价格:¥2800.0
    产品编号bs-15483R
    英文名称HIBADH Rabbit pAb
    中文名称3-羟基异丁酸脱氢酶抗体
    英文别名3 hydroxy 2 methylpropanoate:NAD(+) oxidoreductase; 3 hydroxyisobutyrate dehydrogenase; 3 hydroxyisobutyrate dehydrogenase mitochondrial; EC 1.1.1.31; MGC40361; NS5ATP1; 3HIDH_HUMAN.
    产品应用WB=1:500-2000

    Not yet tested in other applications.
    Optimal working dilutions must be determined by the end user.

    交叉反应Human, Mouse (Rat, Cow, Horse, Sheep)
    抗体来源Rabbit
    免疫原KLH conjugated synthetic peptide derived from human HIBADH
    亚型IgG
    性状Liquid
    纯化方法affinity purified by Protein A
    克隆类型Polyclonal
    理论分子量32 kDa
    浓度1mg/ml
    储存液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    研究领域

    Cell Biology > Other Antibodies > Oxidative Stress

    Metabolism > Pathways and Processes > Metabolic signaling pathways > Amino acid metabolism

    Metabolism > Pathways and Processes > Mitochondrial Metabolism > Mitochondrial markers

    Metabolism > Pathways and Processes > Redox metabolism > Oxidative stress

    Signal Transduction > Metabolism > Amino Acids

    Signal Transduction > Metabolism > Mitochondrial

    亚细胞定位Mitochondrion.
    组织特异性Detected in skin fibroblasts.
    相似性Belongs to the 3-hydroxyisobutyrate dehydrogenase family.
    保存条件Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    背景资料HIBADH is a 336 amino acid mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidization of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7.0 and 10.0, with optimal activity between 8.8 and 9.0. It was previously hypothesized that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterized by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.

     

    应用推荐稀释比例
    {WB}{1:500-2000}

     

    HIBADH Rabbit pAb(bs-15483R)-5
    Sample:
    193T(Human) Cell Lysate at 30 ug
    MCF-7(Human) Cell Lysate at 30 ug
    HepG2(Human) Cell Lysate at 30 ug
    Kidney (Mouse) Lysate at 40 ug
    Primary: Anti- HIBADH (bs-15483R) at 1/1000 dilution
    Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
    Predicted band size: 32 kD
    Observed band size: 30 kD

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