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HIBADH 抗体 (AA 251-336) (AbBy Fluor® 350)

HIBADH 适用: 人, 小鼠 WB, IF (cc), IF (p) 宿主: 兔 Polyclonal AbBy Fluor® 350
产品编号 ABIN1692767
发货至: 中国
  • 抗原 See all HIBADH 抗体
    HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
    抗原表位
    • 14
    • 14
    • 7
    • 4
    • 4
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 251-336
    适用
    • 51
    • 33
    • 11
    • 5
    • 4
    • 4
    • 4
    • 3
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    人, 小鼠
    宿主
    • 49
    • 2
    克隆类型
    • 51
    多克隆
    标记
    • 22
    • 3
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This HIBADH antibody is conjugated to AbBy Fluor® 350
    应用范围
    • 41
    • 20
    • 12
    • 12
    • 7
    • 7
    • 3
    • 3
    • 2
    Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
    交叉反应
    人, 小鼠
    预测反应
    Rat,Cow,Sheep,Horse
    纯化方法
    Purified by Protein A.
    免疫原
    KLH conjugated synthetic peptide derived from human HIBADH
    亚型
    IgG
    Top Product
    Discover our top product HIBADH Primary Antibody
  • 应用备注
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    限制
    仅限研究用
  • 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. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    有效期
    12 months
  • 抗原
    HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
    别名
    Hibadh (HIBADH 产品)
    别名
    NS5ATP1 antibody, 6430402H10Rik antibody, AI265272 antibody, hibadh antibody, zgc:66262 antibody, wu:fb07f02 antibody, zgc:100804 antibody, 3-hydroxyisobutyrate dehydrogenase antibody, 3-hydroxyisobutyrate dehydrogenase b antibody, 3-hydroxyisobutyrate dehydrogenase S homeolog antibody, 3-hydroxyisobutyrate dehydrogenase a antibody, HIBADH antibody, Hibadh antibody, hibadhb antibody, hibadh.S antibody, hibadha antibody
    背景

    Synonyms: 3 hydroxy 2 methylpropanoate:NAD+ oxidoreductase, 3 hydroxyisobutyrate dehydrogenase, 3 hydroxyisobutyrate dehydrogenase mitochondrial, EC 1.1.1.31, MGC40361, NS5ATP1, 3HIDH_HUMAN.

    Background: 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.

    基因ID
    11112
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