Your Good Partner in Biology Research

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

TMLHE Antibody

  • 货号：
    CSB-PA023908LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western blot
      All lanes: TMLHE antibody at 2µg/ml + MCF-7 whole cell lysate
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 50, 47, 39, 45, 43, 42, 51 kDa
      Observed band size: 50 kDa
    • Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA023908LA01HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human heart tissue using CSB-PA023908LA01HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) TMLHE Polyclonal antibody
  • Uniprot No.：
    Q9NVH6
  • 基因名：
    TMLHE
  • 别名：
    AUTSX6 antibody; BBOX2 antibody; Butyrobetaine (gamma) 2 oxoglutarate dioxygenase (gamma butyrobetaine hydroxylase) 2 antibody; Epsilon-trimethyllysine 2-oxoglutarate dioxygenase antibody; Epsilon-trimethyllysine hydroxylase antibody; FLJ10727 antibody; TML dioxygenase antibody; TML hydroxylase antibody; TML-alpha-ketoglutarate dioxygenase antibody; TMLD antibody; TMLH antibody; TMLH_HUMAN antibody; TMLHE antibody; TMLHED antibody; Trimethyllysine dioxygenase, mitochondrial antibody; Trimethyllysine hydroxylase epsilon antibody; XAP130 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Trimethyllysine dioxygenase, mitochondrial protein (16-376AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，TMLHE Antibody (CSB-PA023908LA01HU)，的标记方式是Non-conjugated。对于TMLHE 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-PA023908LB01HU TMLHE Antibody, HRP conjugated ELISA
    FITC CSB-PA023908LC01HU TMLHE Antibody, FITC conjugated
    Biotin CSB-PA023908LD01HU TMLHE 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, WB, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:20-1:200
  • 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：
    Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML).
  • 基因功能参考文献：
    1. Case Report: complex recombination with deletion in the F8 and duplication in the TMLHE mediated by int22h copies during early embryogenesis in proband's mother. PMID: 28492696
    2. Study found 3 mutations in TMLHE to be associated with autism spectrum disorder, c.229C>T/p.Arg77X, c.730G>C/p.Asp244His, and c.1107G>T/p.Glu369Asp. PMID: 23092983
    3. TMLHE deficiency is common in control males and was not significantly increased in frequency in probands from simplex autism families, however, it was 2.82-fold more frequent in probands from male-male multiplex autism families. PMID: 22566635
    4. Use of array CGH to detect exonic copy number variants throughout the genome in autism families detects a novel deletion in TMLHE. PMID: 21865298
    5. C-terminal region of trimethyllysine hydroxylase, epsilon contains the main determinants for its enzymatic activity including a key H389 residue PMID: 15754339
    6. By 5' and 3' RACE, we identified and mapped two alternative 5' TMLH first exons and seven alternative 3'-splice variants. PMID: 17408883

    显示更多

    收起更多

  • 相关疾病：
    Autism, X-linked 6 (AUTSX6)
  • 亚细胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Gamma-BBH/TMLD family
  • 组织特异性：
    All isoforms, but isoform 8, are widely expressed in adult and fetal tissues. Isoform 8 is restricted to heart and skeletal muscle.
  • 数据库链接：

    HGNC: 18308

    OMIM: 300777

    KEGG: hsa:55217

    STRING: 9606.ENSP00000335261

    UniGene: Hs.133321