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LIAS Antibody

  • 中文名稱：
    LIAS兔多克隆抗體
  • 貨號：
    CSB-PA012927GA01HU
  • 規(guī)格：
    ￥3,900
  • 其他：

產(chǎn)品詳情

  • Uniprot No.：
  • 基因名：
    LIAS
  • 別名：
    LIAS antibody; LAS antibody; HUSSY-01Lipoyl synthase antibody; mitochondrial antibody; EC 2.8.1.8 antibody; Lipoate synthase antibody; LS antibody; Lip-syn antibody; Lipoic acid synthase antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human,Mouse,Rat
  • 免疫原：
    Human LIAS
  • 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(chǎn)品提供形式：
    Liquid
  • 應用范圍：
    ELISA,WB,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.

產(chǎn)品評價

靶點詳情

  • 功能：
    Catalyzes the radical-mediated insertion of two sulfur atoms into the C-6 and C-8 positions of the octanoyl moiety bound to the lipoyl domains of lipoate-dependent enzymes, thereby converting the octanoylated domains into lipoylated derivatives.
  • 基因功能參考文獻：
    1. this study used a bioinformatics approach to predict its structure. . A homology model for LIAS protein was generated using X-ray crystallographic structure of Thermosynechococcus elogatsu. The active site of LIAS protein was mapped and docked with S-Adenosyl Methionine PMID: 27717843
    2. oxoglutarate dehydrogenase (OGDH) and lipoic acid synthase (LIAS), which when mutated stabilize HIF1alpha in a non-hydroxylated form. PMID: 27923773
    3. heterozygous mutations (c.738-2A>G and c.929T>C (p.Met310Thr)) in LIAS. PMID: 26108146
    4. Patients with LIAS nonketotic hyperglycinemia varied in disease severity and cortical involvement. PMID: 24334290
    5. We identified the homozygous mutation c.746G>A (p.Arg249His) in LIAS in an individual with neonatal-onset epilepsy, muscular hypotonia, lactic acidosis, and elevated glycine concentration in plasma and urine PMID: 22152680
    6. Lipoic acid synthetase deficiency results in an overall disturbance in the antioxidant defense network, leading to increased inflammation, insulin resistance, and mitochondrial dysfunction. PMID: 19074983

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  • 相關(guān)疾?。?/div>
    Hyperglycinemia, lactic acidosis, and seizures (HGCLAS)
  • 亞細胞定位：
    Mitochondrion.
  • 蛋白家族：
    Radical SAM superfamily, Lipoyl synthase family
  • 數(shù)據(jù)庫鏈接：

    HGNC: 16429

    OMIM: 607031

    KEGG: hsa:11019

    STRING: 9606.ENSP00000261434

    UniGene: Hs.550502