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  4. Recombinant Human 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial (HIBCH)

Recombinant Human 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial (HIBCH)

  • 中文名称：
    人HIBCH重组蛋白
  • 货号：
    CSB-YP747360HU
  • 规格：
  • 来源：
    Yeast
  • 其他：
  • 中文名称：
    人HIBCH重组蛋白
  • 货号：
    CSB-EP747360HU
  • 规格：
  • 来源：
    E.coli
  • 其他：
  • 中文名称：
    人HIBCH重组蛋白
  • 货号：
    CSB-EP747360HU-B
  • 规格：
  • 来源：
    E.coli
  • 共轭：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 中文名称：
    人HIBCH重组蛋白
  • 货号：
    CSB-BP747360HU
  • 规格：
  • 来源：
    Baculovirus
  • 其他：
  • 中文名称：
    人HIBCH重组蛋白
  • 货号：
    CSB-MP747360HU
  • 规格：
  • 来源：
    Mammalian cell
  • 其他：

产品详情

  • 纯度：
    >85% (SDS-PAGE)
  • 基因名：
    HIBCH
  • Uniprot No.：
    Q6NVY1
  • 别名：
    3 hydroxyisobutyryl Coenzyme A hydrolase ; 3 hydroxyisobutyryl Coenzyme A hydrolase; mitochondrial ; 3-hydroxyisobutyryl-CoA hydrolase; 3-hydroxyisobutyryl-coenzyme A hydrolase; BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE; HIB CoA hydrolase; HIB-CoA hydrolase; HIBCH; HIBCH_HUMAN; HIBCoA hydrolase; HIBYL CoA H; HIBYL CoAH; HIBYL-CoA-H; HIBYLCoA H; HIBYLCoAH; mitochondrial
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Full Length of Mature Protein
  • 表达区域：
    33-386
  • 氨基酸序列
    DAAEEVLL EKKGCTGVIT LNRPKFLNAL TLNMIRQIYP QLKKWEQDPE TFLIIIKGAG GKAFCAGGDI RVISEAEKAK QKIAPVFFRE EYMLNNAVGS CQKPYVALIH GITMGGGVGL SVHGQFRVAT EKCLFAMPET AIGLFPDVGG GYFLPRLQGK LGYFLALTGF RLKGRDVYRA GIATHFVDSE KLAMLEEDLL ALKSPSKENI ASVLENYHTE SKIDRDKSFI LEEHMDKINS CFSANTVEEI IENLQQDGSS FALEQLKVIN KMSPTSLKIT LRQLMEGSSK TLQEVLTMEY RLSQACMRGH DFHEGVRAVL IDKDQSPKWK PADLKEVTEE DLNNHFKSLG SSDLKF
  • 蛋白标签：
    Tag type will be determined during the manufacturing process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 产品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 复溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

产品评价

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HIBCH antibodies

靶点详情

  • 功能：
    Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.
  • 基因功能参考文献：
    1. Novel (founder) mutation in HIBCH that causes a mild phenotype, allowing survival into adulthood. PMID: 27400804
    2. Polymorphism in HIBCH is associated with HIBCH deficiency. PMID: 27132595
    3. findings demonstrated a novel homozygous pathogenic missense mutation c.950G PMID: 24299452
    4. Molecular analysis in both patients uncovered mutations in the HIBCH gene, including one missense mutation in a conserved part of the protein and two mutations affecting splicing. PMID: 17160907
  • 相关疾病：
    3-hydroxyisobutryl-CoA hydrolase deficiency (HIBCHD)
  • 亚细胞定位：
    Mitochondrion.
  • 蛋白家族：
    Enoyl-CoA hydratase/isomerase family
  • 组织特异性：
    Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung.
  • 数据库链接：

    HGNC: 4908

    OMIM: 250620

    KEGG: hsa:26275

    STRING: 9606.ENSP00000352706

    UniGene: Hs.656685

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