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  4. HADH Monoclonal Antibody

HADH Monoclonal Antibody

  • 中文名稱：
    HADH Monoclonal Antibody
  • 貨號：
    CSB-MA971598
  • 規格：
    ￥1320
  • 圖片：
    • Immunohistochemistry was performed on paraffin-embedded human breast carcinoma using HADH antibody. Antigen retrieval was done in sodium citrate buffer (pH 6.0). DAB was used for detection, with hematoxylin counterstaining. Images were acquired using a Nikon Ci-L Plus microscope (40× objective). Scale bar: 25 μm.
    • Western blotting analysis using HADH antibody. Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with HADH antibody and HRP-conjugated goat anti-mouse secondary antibody respectively.
    • Western blotting analysis using HADH antibody. HADH expression in wild type (WT) and HADH knockout (KO) HeLa cells with 20 μg of total cell lysates. Hsp90 α serves as a loading control. The blot was incubated with HADH antibody and HRP-conjugated goat anti-mouse secondary antibody respectively.
  • 其他：

產品詳情

  • 產品名稱：
    Mouse anti-Homo sapiens (Human) HADH Monoclonal antibody
  • Uniprot No.：
  • 基因名：
    HADH
  • 別名：
    HADH; Hydroxyacyl-CoA Dehydrogenase; SCHAD; HADHSC; HADH1; Medium And Short-Chain L-3-Hydroxyacyl-Coenzyme A Dehydrogenase; L-3-Hydroxyacyl-Coenzyme A Dehydrogenase, Short Chain; Hydroxyacyl-Coenzyme A Dehydrogenase, Mitochondrial; Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase; EC 1.1.1.35; HCDH; HAD; Testis Secretory Sperm-Binding Protein Li 203a; Hydroxyacyl-Coenzyme A Dehydrogenase; EC 1.1.1; MSCHAD; HHF4; HAD1
  • 宿主：
    Mouse
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human HADH protein
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated
  • 克隆類型：
    Monoclonal
  • 抗體亞型：
    Mouse IgG2b
  • 純化方式：
    Affinity-chromatography
  • 克隆號：
    24E4
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    PBS (pH 7.4) containing 50% glycerol, and 0.02% sodium azide.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA, WB, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:400-1:2000
    IHC 1:100-1:200
  • Protocols：
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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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  • 相關疾病：
    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