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PITRM1 Antibody

  • 货号：
    CSB-PA018041GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q5JRX3
  • 基因名：
  • 别名：
    hMP1 antibody; hPreP antibody; KIAA1104 antibody; Metalloprotease 1 antibody; MGC138192 antibody; MGC141929 antibody; mitochondrial antibody; MP1 antibody; Pitrilysin metallopeptidase 1 antibody; Pitrilysin metalloproteinase 1 antibody; PITRM 1 antibody; PITRM1 antibody; PreP antibody; PreP peptidasome antibody; PREP_HUMAN antibody; Presequence protease antibody; Presequence protease mitochondrial antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human PITRM1
  • 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
  • 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：
    Metalloendopeptidase of the mitochondrial matrix that functions in peptide cleavage and degradation rather than in protein processing. Has an ATP-independent activity. Specifically cleaves peptides in the range of 5 to 65 residues. Shows a preference for cleavage after small polar residues and before basic residues, but without any positional preference. Degrades the transit peptides of mitochondrial proteins after their cleavage. Also degrades other unstructured peptides. It is also able to degrade amyloid-beta protein 40, one of the peptides produced by APP processing, when it accumulates in mitochondrion. It is a highly efficient protease, at least toward amyloid-beta protein 40. Cleaves that peptide at a specific position and is probably not processive, releasing digested peptides intermediates that can be further cleaved subsequently.
  • 基因功能参考文献：
    1. Redox control of hPreP in the mitochondrial matrix and the protective role of the conserved methionine 206 residue as an internal antioxidant. PMID: 23041349
    2. The s identified two siblings carrying a homozygous PITRM1 missense mutation (c.548G>A, p.Arg183Gln) associated with an autosomal recessive, slowly progressive syndrome characterised by mental retardation, spinocerebellar ataxia, cognitive decline and psychosis. PMID: 26697887
    3. Data show that the hPreP presequence only targets GFP to the matrix of mammalian and yeast mitochondria. PMID: 21621546
    4. This study demonstrated decreased proteolytic activity of the mitochondrial amyloid-beta degrading enzyme, PreP peptidasome, in Alzheimer's disease brain mitochondria. PMID: 21750375
    5. our data suggest that genetic variation in the hPreP gene PITRM1 may potentially contribute to mitochondrial dysfunctions PMID: 19962426
    6. The substrate specificity of the mitochondrial metallopeptidase proteinase 1 (MP1) was investigated and its mitochondrial targeting signal identified. PMID: 19196155
    7. Data revealed that six polymorphisms of F10, PITRM1, PCSK2, JPH3, MYO7B, and AKAP12 were related (P<0.05) to the prevalence of chronic kidney disease. PMID: 19724895

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  • 亚细胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Peptidase M16 family, PreP subfamily
  • 组织特异性：
    Widely expressed. Expressed at higher level in muscle and heart compared to brain, pancreas, liver, lung and placenta.
  • 数据库链接：

    HGNC: 17663

    KEGG: hsa:10531

    STRING: 9606.ENSP00000370377

    UniGene: Hs.528300