Your Good Partner in Biology Research

  1. Home
  2. 抗体
  3. 多克隆抗体
  4. IVD Antibody

IVD Antibody

  • 货号：
    CSB-PA011921LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • IHC image of CSB-PA011921LA01HU diluted at 1:500 and staining in paraffin-embedded human appendix tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) IVD Polyclonal antibody
  • Uniprot No.：
    P26440
  • 基因名：
    IVD
  • 别名：
    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
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Isovaleryl-CoA dehydrogenase, mitochondrial protein (139-241AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，IVD Antibody (CSB-PA011921LA01HU)，的标记方式是Non-conjugated。对于IVD Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA011921LB01HU IVD Antibody, HRP conjugated ELISA
    FITC CSB-PA011921LC01HU IVD Antibody, FITC conjugated
    Biotin CSB-PA011921LD01HU IVD Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:500-1:1000
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献：
    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

    显示更多

    收起更多

  • 相关疾病：
    Isovaleric acidemia (IVA)
  • 亚细胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Acyl-CoA dehydrogenase family
  • 数据库链接：

    HGNC: 6186

    OMIM: 243500

    KEGG: hsa:3712

    STRING: 9606.ENSP00000418397

    UniGene: Hs.513646