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ACADSB Antibody

  • 中文名称：
    ACADSB兔多克隆抗体
  • 货号：
    CSB-PA001128GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    P45954
  • 基因名：
    ACADSB
  • 别名：
    2 MEBCAD antibody; 2 methyl branched chain acyl CoA dehydrogenase antibody; 2 methylbutyryl CoA dehydrogenase antibody; 2 methylbutyryl coenzyme A dehydrogenase antibody; 2-MEBCAD antibody; 2-methyl branched chain acyl-CoA dehydrogenase antibody; 2-methylbutyryl-CoA dehydrogenase antibody; 2-methylbutyryl-coenzyme A dehydrogenase antibody; ACAD7 antibody; ACADSB antibody; ACDSB_HUMAN antibody; acyl CoA dehydrogenase; short/branched chain antibody; acyl Coenzyme A dehydrogenase short branched chain antibody; mitochondrial antibody; OTTHUMP00000020685 antibody; OTTHUMP00000046795 antibody; SBCAD antibody; Short/branched chain specific acyl-CoA dehydrogenase antibody; short/branched chain specific acyl-CoA dehydrogenase; mitochondrial antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human ACADSB
  • 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：
    Short and branched chain specific acyl-CoA dehydrogenase that catalyzes the removal of one hydrogen from C-2 and C-3 of the fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, acts specifically on short and branched chain acyl-CoA derivatives such as (S)-2-methylbutyryl-CoA as well as short straight chain acyl-CoAs such as butyryl-CoA. Plays an important role in the metabolism of L-isoleucine by catalyzing the dehydrogenation of 2-methylbutyryl-CoA, one of the steps of the L-isoleucine catabolic pathway. Can also act on valproyl-CoA, a metabolite of valproic acid, an antiepileptic drug.
  • 基因功能参考文献：
    1. the c.1165 A>G mutation before knowing whether the optimal screening cut-off would minimize true positives or false negatives for SBCADD associated with this mutation. PMID: 23712021
    2. Strong candidate gene for mitochondrial disease, based on recessive mutations detected in infantile patients PMID: 22277967
    3. These findings confirm that SBCAD deficiency can be identified through newborn screening by acylcarnitine analysis. PMID: 20547083
    4. Differences between the rat and human enzyme at positions 383, 222, and 220 alter substrate specificity without affecting substrate binding. PMID: 12855692
    5. Identification and characterization of an IVS3+3A>G mutation (c.303+3A>G) in the SBCAD gene, and provide evidence that this mutation is disease-causing. PMID: 16317551
    6. Results examine the mechanistic basis for dysfunction of the common variant short-chain acyl-CoA dehydrogenases and demonstrate that mutations can have a large impact on the redox properties of the enzyme. PMID: 16331963
    7. Results indicate that substrate redox activation occurs in short-chain acyl-CoA dehydrogenase leading to a large enzyme midpoint potential shift. PMID: 16331964

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  • 相关疾病：
    Short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD)
  • 亚细胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Acyl-CoA dehydrogenase family
  • 组织特异性：
    Ubiquitously expressed.
  • 数据库链接：

    HGNC: 91

    OMIM: 600301

    KEGG: hsa:36

    STRING: 9606.ENSP00000357873

    UniGene: Hs.81934