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PPT1 Antibody

  • 货号：
    CSB-PA030196
  • 规格：
    ￥880
  • 其他：

产品详情

  • Uniprot No.：
    P50897
  • 基因名：
  • 别名：
    Ceroid palmitoyl palmitoyl protein thioesterase 1 antibody; CLN1 antibody; EC 3.1.2.22 antibody; INCL antibody; Palmitoyl protein hydrolase 1 antibody; Palmitoyl protein thioesterase 1 antibody; Palmitoyl-protein hydrolase 1 antibody; Palmitoyl-protein thioesterase 1 antibody; PPT antibody; PPT-1 antibody; PPT1 antibody; PPT1_HUMAN antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Synthesized peptide derived from the N-terminal region of Human CLN1.
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 产品提供形式：
    Liquid
  • 应用范围：
    IHC, ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:100-1:300
    ELISA 1:10000
  • 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：
    Removes thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons.
  • 基因功能参考文献：
    1. Targeting PPT1 blocks mTOR signaling in a manner distinct from catalytic inhibitors, while concurrently inhibiting autophagy, thereby providing a new strategy for cancer therapy. PMID: 28899863
    2. the combination of elevated glycolysis and deficient MRPL13 activity was closely linked to CLN1-mediated tumor activity in human hepatoma cells PMID: 28978646
    3. Proteomics analysis on isolated cilia revealed 660 proteins, which differed in their abundance levels between wild type and Ppt1 knock out. PMID: 28334871
    4. we reveal the existence of a positive feedback loop, where palmitoylation of PPT1 results in decreased activity and subsequent elevation in the amount of palmitoylated proteins. PMID: 26731412
    5. analysis of the palmitoyl protein thioesterase 1 interactome in SH-SY5Y human neuroblastoma cells PMID: 25865307
    6. Data (including data from knockout mice) suggest that deficiency of PPT1 leads to accumulation of granular osmiophilic deposits in many cell types, especially in astrocytes. [review-like article] PMID: 25233404
    7. Data suggest that human monocytes and macrophages express PPT1; PPT1 appears to contribute 32-40% of 2-arachidonylglycerol hydrolysis activity in THP1 monocyte cell line. PMID: 24083319
    8. This neuroimaging finding in PPT1-related neuronal ceroid lipofuscinosis was not previously reported. PMID: 22520356
    9. Stop codon read-through with PTC124 induces palmitoyl-protein thioesterase-1 activity, reduces thioester load and suppresses apoptosis in cultured cells from Infantile neuronal ceroid lipofuscinosis patients. PMID: 21704547
    10. Results describe the correlation between the three-dimensional structural changes in mutant palmitoyl protein thioesterase 1 and biochemical phenotypes. PMID: 19793631
    11. mutated in neuronal ceroid lipofuscinosis PMID: 12025857
    12. The clinical, biochemical, and molecular genetic aspects of lysosomal storage disorders are discussed in this review PMID: 12125808
    13. The crystal structure of palmitoyl protein thioesterase-2 (PPT2) reveals the basis for divergent substrate specificities of the two lysosomal thioesterases, PPT1 and PPT2. PMID: 12855696
    14. there is a close correlation between CLN2 and CLN1 expression and colorectal carcinoma progression and metastasis and suggest that they may be potential molecular targets PMID: 16518810
    15. Results show that PPT1-deficiency causes a defect in fluid-phase and receptor-mediated endocytosis. PMID: 16542649
    16. ER stress due to PPT1-deficiency increases ROS and disrupts calcium homeostasis activating caspase-9 and caspase-9 activation mediates caspase-3 activation and apoptosis contributing to rapid neurodegeneration in INCL. PMID: 16571600
    17. Adult neuronal ceroid lipofuscinosis caused by deficiency in palmitoyl protein thioesterase 1. PMID: 17261688
    18. Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals in humans and mice PMID: 18704195
    19. study presents clinical and diagnostic investigations in six children with variant late infantile neuronal ceroid lipofuscinosis and mutations in CLN1 including a new large-scale deletion on one allele PMID: 19302939
    20. Using shotgun mass spectrometry, we found this protein differentially expressed in the dorsolateral prefrontal cortex from patients with schizophrenia. PMID: 19165527
    21. The function of PPT1 is to hydrolyze long-chain fatty acids from cysteine residues of fatty acylated proteins in the lysosome. PMID: 8816748

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  • 相关疾病：
    Ceroid lipofuscinosis, neuronal, 1 (CLN1)
  • 亚细胞定位：
    Lysosome. Secreted.
  • 蛋白家族：
    Palmitoyl-protein thioesterase family
  • 数据库链接：

    HGNC: 9325

    OMIM: 256730

    KEGG: hsa:5538

    STRING: 9606.ENSP00000394863

    UniGene: Hs.3873