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IVD Antibody, HRP conjugated

  • 中文名稱：
    IVD兔多克隆抗體, HRP偶聯(lián)
  • 貨號(hào)：
    CSB-PA011921LB01HU
  • 規(guī)格：
    ￥880
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) IVD Polyclonal antibody
  • 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(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human Isovaleryl-CoA dehydrogenase, mitochondrial protein (139-241AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    HRP
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA
  • Protocols：
  • 儲(chǔ)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.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)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(xiàn)：
    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(guān)疾病：
    Isovaleric acidemia (IVA)
  • 亞細(xì)胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Acyl-CoA dehydrogenase family
  • 數(shù)據(jù)庫(kù)鏈接：

    HGNC: 6186

    OMIM: 243500

    KEGG: hsa:3712

    STRING: 9606.ENSP00000418397

    UniGene: Hs.513646