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AUH Antibody

  • 货号：
    CSB-PA002451GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q13825
  • 基因名：
    AUH
  • 别名：
    AUHMethylglutaconyl-CoA hydratase antibody; mitochondrial antibody; EC 4.2.1.18 antibody; AU-specific RNA-binding enoyl-CoA hydratase antibody; AU-binding protein/enoyl-CoA hydratase antibody; Itaconyl-CoA hydratase antibody; EC 4.2.1.56 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human AUH
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • 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 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA. Also has itaconyl-CoA hydratase activity by converting itaconyl-CoA into citramalyl-CoA in the C5-dicarboxylate catabolism pathway. The C5-dicarboxylate catabolism pathway is required to detoxify itaconate, a vitamin B12-poisoning metabolite. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs.
  • 基因功能参考文献：
    1. AUH localizes to the inner mitochondrial membrane and matrix where it associates with mitochondrial ribosomes and regulates protein synthesis. PMID: 24598254
    2. Phenotypic heterogeneity in two siblings with 3-methylglutaconic aciduria type I caused by a novel deletion of exons 1-3 within the AUH gene. PMID: 21840233
    3. 3-Methylglutaconic aciduria type I is caused by mutations in AUH PMID: 12434311
    4. Human 3-methylglutaconyl-CoA hydratase is identical with RNA-binding protein (AUH); molecular analyses of MGA1 patients show homozygosity or compound heterozygosity for mutations in AUH. PMID: 12655555
    5. Mutation analysis in the AUH gene revealed homozygosity for a novel splice site mutation IVS9-2A>G. We conclude that MGA1 may be associated with fever-associated seizures even in children without delayed psychomotor development. PMID: 15033206
    6. Mutations in the AUH gene are linked to metabolic disease 3-methylglutaconic aciduria type I (MGA1). PMID: 16640564
    7. The AUH trimer dimerizes upon binding to one molecule of a long RNA containing 24 repeats of the AUUU motif, (AUUU)(24)A. PMID: 18831052

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  • 相关疾病：
    3-methylglutaconic aciduria 1 (MGA1)
  • 亚细胞定位：
    Mitochondrion.
  • 蛋白家族：
    Enoyl-CoA hydratase/isomerase family
  • 数据库链接：

    HGNC: 890

    OMIM: 250950

    KEGG: hsa:549

    STRING: 9606.ENSP00000364883

    UniGene: Hs.175905