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IVD Antibody

  • 中文名稱:
    IVD兔多克隆抗體
  • 貨號:
    CSB-PA011921GA01HU
  • 規格:
    ¥3,900
  • 其他:

產品詳情

  • Uniprot No.:
  • 基因名:
    IVD
  • 別名:
    ACAD2 antibody; FLJ12715 antibody; FLJ34849 antibody; Isovaleryl CoA dehydrogenase antibody; Isovaleryl CoA dehydrogenase, mitochondrial antibody; Isovaleryl Coenzyme A dehydrogenase antibody; Isovaleryl-CoA dehydrogenase antibody; IVD antibody; IVD_HUMAN antibody; mitochondrial antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human,Mouse,Rat
  • 免疫原:
    Human IVD
  • 免疫原種屬:
    Homo sapiens (Human)
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen Affinity Purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA,IHC
  • 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:
    Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway. To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as pentanoyl-CoA, hexenoyl-CoA and butenoyl-CoA.
  • 基因功能參考文獻:
    1. Nine novel isovaleryl-CoA dehydrogenase mutations have been found in a Spanish cohort with isovaleric acidemia. PMID: 27904153
    2. Our results have illustrated the heterogeneous mutation spectrum and clinical presentation of IVA in the Japanese patients PMID: 26018748
    3. kinetics and ligand binding of isovaleryl-CoA dehydrogenase PMID: 25450250
    4. study reports the first Saudi isovaleric acidemia patients from a consanguineous family with a novel transversion (p.G362V) and briefly discuss likely phenotype-genotype correlation of the disease in the Saudi population PMID: 23063737
    5. A heterogeneous mutation spectrum in the IVD gene was identified in isovaleric acidemia patients in the United Arab Emirates. PMID: 22960500
    6. All were homozygous for a single c.367 G > A (p.G123R) mutation. Despite the genetic homogeneity of this South African group, the clinical presentation varied, ranging from mental handicap and episodes of metabolic derangement to an asymptomatic state PMID: 22350545
    7. A child with isovaleric acidemia was subsequently found to harbour a known missense mutation (c.A1199G [p.Y371C]) and a novel 4-bp duplication (c.1148_1151dupGCTA [p.Y355X]) in the IVD gene. The former may be a founder mutation in the Chinese population. PMID: 20519759
    8. Replacement of the catalytic glutamate in either short-chain acyl-CoA dehydrogenase (SCAD) or isovaleryl-CoA dehydrogenase (IVD)with glycine resulted in a several-fold reduction in affinity for substrate. PMID: 16376132
    9. Mutations of isovaleryl-CoA dehydrogenase gene is associated with isovaleric acidemia PMID: 17576084

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  • 相關疾病:
    Isovaleric acidemia (IVA)
  • 亞細胞定位:
    Mitochondrion matrix.
  • 蛋白家族:
    Acyl-CoA dehydrogenase family
  • 數據庫鏈接:

    HGNC: 6186

    OMIM: 243500

    KEGG: hsa:3712

    STRING: 9606.ENSP00000418397

    UniGene: Hs.513646



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