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  4. Recombinant Human Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial (HADH)

Recombinant Human Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial (HADH)

  • 中文名稱：
    人HADH重組蛋白
  • 貨號：
    CSB-YP614992HU
  • 規格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    人HADH重組蛋白
  • 貨號：
    CSB-EP614992HU
  • 規格：
  • 來源：
    E.coli
  • 其他：
  • 中文名稱：
    人HADH重組蛋白
  • 貨號：
    CSB-EP614992HU-B
  • 規格：
  • 來源：
    E.coli
  • 共軛：
    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：
  • 中文名稱：
    人HADH重組蛋白
  • 貨號：
    CSB-BP614992HU
  • 規格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    人HADH重組蛋白
  • 貨號：
    CSB-MP614992HU
  • 規格：
  • 來源：
    Mammalian cell
  • 其他：

產品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
    HADH
  • Uniprot No.：
  • 別名：
    3 hydroxyacyl Coenzyme A dehydrogenase; HAD; HADH; HADH1; HADHSC; HADHSC, formerly; HADSC, formerly; HCDH; HCDH_HUMAN; HHF4; Hydroxyacyl CoA dehydrogenase; Hydroxyacyl-coenzyme A dehydrogenase; hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; L 3 hydroxyacyl Coenzyme A dehydrogenase short chain; M SCHAD; Medium and short chain L 3 hydroxyacyl coenzyme A dehydrogenase ; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; MGC8392; mitochondrial; MSCHAD; OTTHUMP00000162626; OTTHUMP00000219688; SCHAD; SCHAD, formerly; Short chain 3 hydroxyacyl CoA dehydrogenase mitochondrial; short chain 3-hydroxyacyl-coa dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Full Length of Mature Protein
  • 表達區域：
    13-314
  • 氨基酸序列
    SSSSTASA SAKKIIVKHV TVIGGGLMGA GIAQVAAATG HTVVLVDQTE DILAKSKKGI EESLRKVAKK KFAENLKAGD EFVEKTLSTI ATSTDAASVV HSTDLVVEAI VENLKVKNEL FKRLDKFAAE HTIFASNTSS LQITSIANAT TRQDRFAGLH FFNPVPVMKL VEVIKTPMTS QKTFESLVDF SKALGKHPVS CKDTPGFIVN RLLVPYLMEA IRLYERGDAS KEDIDTAMKL GAGYPMGPFE LLDYVGLDTT KFIVDGWHEM DAENPLHQPS PSLNKLVAEN KFGKKTGEGF YKYK
  • 蛋白標簽：
    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.
  • 產品提供形式：
    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.
  • 復溶：
    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.
  • 儲存條件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質期：
    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.
  • 貨期：
    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.
  • 注意事項：
    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.

產品評價

靶點詳情

  • 功能：
    Mitochondrial fatty acid beta-oxidation enzyme that catalyzes the third step of the beta-oxidation cycle for medium and short-chain 3-hydroxy fatty acyl-CoAs (C4 to C10). Plays a role in the control of insulin secretion by inhibiting the activation of glutamate dehydrogenase 1 (GLUD1), an enzyme that has an important role in regulating amino acid-induced insulin secretion.
  • 基因功能參考文獻：
    1. The most frequently seen mutations in Turkish patients with congenital hyperinsulinism (CHI) were ATP binding cassette subfamily C member 8 (ABCC8) gene, followed by 3-hydroxyacyl CoA dehydrogenase (HADH) and kcnj11 channel (KCNJ11) genes. PMID: 27181376
    2. Paretic muscle in hemiparetic stroke survivors had lower HAD concentration. PMID: 26361074
    3. We present clinical and laboratory findings together with the long-term clinical course of a case with a deep intronic HADH splicing mutation (c.636+471G>T) causing neonatal-onset hyperinsulinemic hypoglycemia with mild progression PMID: 26316438
    4. in a cohort of hyperinsulinemic hypoglycemia patients from Isfahan, Iran, 78% were noted to have disease-causing mutations: 48% had HADH mutations and 26% had ABCC8 mutations. PMID: 26268944
    5. Next-generation sequencing reveals deep intronic cryptic ABCC8 and HADH splicing founder mutations causing hyperinsulinism by pseudoexon activation. PMID: 23273570
    6. Loss of function mutations in 3-Hydroxyacyl-CoA Dehydrogenase (HADH) cause leucine sensitive hyperinsulinaemic hypoglycaemia. PMID: 22583614
    7. Clinical, biochemical and molecular findings of four new Caucasian patients with HADH deficiency. PMID: 21347589
    8. We recommend that HADH sequence analysis is considered in all patients with diazoxide-responsive hyperinsulinemic hypoglycemia when recessive inheritance is suspected PMID: 21252247
    9. Congenital hyperinsulinism due to mutations in HNF4A and HADH. PMID: 20931292
    10. SCHAD deficiency can result in persistent hyperinsulinemic hypoglycemia of infancy PMID: 14693719
    11. Unlikely that variation in HADHSC plays a major role in the pathogenesis of type 2 diabetes in the examined cohorts. PMID: 17065362
    12. This case indicates that mutations of the HADH gene should be sought in hyperinsulinemic patients in whom diffuse form of hyperinsulinemic hypoglycemia. PMID: 19318379
    13. To investigate its function in this catalytic dyad, Glu(170) was replaced with glutamine (E170Q), and the mutant enzyme was characterized. Substrate and cofactor binding were unaffected by the mutation; E170Q exhibited diminished catalytic activity PMID: 11451959
    14. Patients with the G1528C mutation of 3-hyroxyacyl-CoA dehydrogenase exhibit hepatomegaly and steatosis of the liver, as well as accumulation of fat in the myocardium, renal tubules, and skeletal muscle PMID: 9185222

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  • 相關疾?。?/div>
    3-alpha-hydroxyacyl-CoA dehydrogenase deficiency (HADH deficiency); Familial hyperinsulinemic hypoglycemia 4 (HHF4)
  • 亞細胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    3-hydroxyacyl-CoA dehydrogenase family
  • 組織特異性：
    Expressed in liver, kidney, pancreas, heart and skeletal muscle.
  • 數據庫鏈接：

    HGNC: 4799

    OMIM: 231530

    KEGG: hsa:3033

    UniGene: Hs.438289



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