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LONP1 Antibody

  • 货号：
    CSB-PA958239
  • 规格：
    ￥1100
  • 图片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human breast cancer tissue using CSB-PA958239(LONP1 Antibody) at dilution 1/25, on the right is treated with synthetic peptide. (Original magnification: ×200)
    • The image on the left is immunohistochemistry of paraffin-embedded Human esophagus cancer tissue using CSB-PA958239(LONP1 Antibody) at dilution 1/25, on the right is treated with synthetic peptide. (Original magnification: ×200)
    • Gel: 6%SDS-PAGE,Lysate: 40 μg,Lane 1-5: A431 cells, NIH/3T3 cells, HepG2 cells, Mouse liver tissue, Hela cells,Primary antibody: CSB-PA958239(LONP1 Antibody) at dilution 1/250 dilution,Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution,Exposure time: 30 seconds
  • 其他：

产品详情

  • Uniprot No.：
    P36776
  • 基因名：
    LONP1
  • 别名：
    hLON antibody; hLON ATP dependent protease antibody; LON antibody; lon peptidase 1, mitochondrial antibody; LON protease antibody; Lon protease homolog antibody; Lon protease like protein antibody; Lon protease-like protein antibody; LONHs antibody; LONM_HUMAN antibody; LONP antibody; Lonp1 antibody; MGC1498 antibody; mitochondrial antibody; Mitochondrial ATP dependent protease Lon antibody; Mitochondrial ATP-dependent protease Lon antibody; Mitochondrial lon peptidase 1 antibody; PIM1 antibody; Protease serine 15 antibody; PRSS15 antibody; Serine protease 15 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse
  • 免疫原：
    Synthetic peptide of Human LONP1
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen affinity purification
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:2000-1:5000
    WB 1:500-1:2000
    IHC 1:25-1:100
  • 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：
    ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial promoters and RNA in a single-stranded, site-specific, and strand-specific manner. May regulate mitochondrial DNA replication and/or gene expression using site-specific, single-stranded DNA binding to target the degradation of regulatory proteins binding to adjacent sites in mitochondrial promoters. Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein, helicase Twinkle (TWNK) and the large ribosomal subunit protein bL32m. bL32m is protected from degradation by LONP1 when it is bound to a nucleic acid (RNA), but TWNK is not.
  • 基因功能参考文献：
    1. mitochondrial ATP-dependent Lon protease may serve as a potential biomarker for cancer diagnosis and novel target for the development of anticancer drugs and for predicting of the efficiency and effectiveness of chemotherapy of a variety of cancers. PMID: 29178076
    2. We demonstrate that Lon plays a key role in glioma cell hypoxic survival and mitochondrial respiration, and propose Lon as a promising therapeutic target in the treatment of malignant gliomas. PMID: 27764809
    3. Some features were not consistent with CODAS syndrome but overlapped with Marinesco-Sjogren syndrome, a multisystem disorder caused by a mutation in SIL1. An atypical mutation site may result in atypical presentation of the LONP1 mutation PMID: 28148925
    4. LONP1 function and implication in human aging and disease was reviewed. PMID: 27387767
    5. we observed that Lon protease downregulation is linked to a higher lipofuscinogenesis whereas the application of the mitochondrial-targeted antioxidant mitoTEMPO is able to prevent the accumulation of this protein aggregate. PMID: 28160744
    6. Lon preferentially degrades the phosphorylated subunits of CcO and plays a role in the regulation of CcO activity in hypoxia and ischemia/reperfusion injury. PMID: 28442264
    7. Lon protease (Lonp1), which is a key inductive of mitochondrial unfolded protein response (UPR(mt)) and is required to maintain the mitochondrial quality, was greatly induced in H. pylori infected gastric epithelial cells. PMID: 27108387
    8. This analysis revealed that LONM specifically recognises and degrades unfolded, but not aggregated proteins. PMID: 26627475
    9. Mutations of Lon, which likely impair its chaperone properties, are at the basis of a genetic inherited disease named the cerebral, ocular, dental, auricular, skeletal (CODAS) syndrome. (Review) PMID: 27033304
    10. Inhibition of Lon protease by triterpenoids alters mitochondria and is associated to cell death in human cancer cells. PMID: 26314956
    11. Lon downregulation attenuated hypoxia-induced cardiomyocyte apoptosis through a reduction of reactive oxygen species level. PMID: 25922169
    12. LONP1 encodes an enzyme of bacterial ancestry that participates in protein turnover within the mitochondrial matrix, and mutations in its ATP-binding and proteolytic domains cause CODAS syndrome. PMID: 25808063
    13. A review on the recent discoveries concerning Lon Protease functions. [review] PMID: 26363553
    14. These results suggest that the mechanism underlying cell survival regulated by Lon is mediated by the maintenance of the protein stability of Hsp60-mtHsp70 complex. PMID: 25675302
    15. Silencing of SIRT3 increased the levels of Lon protein and of its acetylation, suggesting that Lon is a target of SIRT3, likely at K917. PMID: 25128872
    16. the structure of human mitochondrial Lon (hLon) protease, is reported. PMID: 25369343
    17. We establish a link between LONP1 and CODAS syndrome in humans. PMID: 25574826
    18. Lonp1 has a protective role against ochratoxin a induced cytotoxicity in kidney cells. PMID: 24565693
    19. StAR proteolysis is executed by at least 2 mitochondrial proteases, the matrix LON protease and the inner membrane complexes of the metalloproteases AFG3L2 and AFG3L2:SPG7/paraplegin. PMID: 24422629
    20. Lon protease deficiency led to an increase in ROS production and to an accumulation of carbonylated protein in the mitochondria. PMID: 24355201
    21. Down-regulation of overexpressed human LON in cervical cancer suppresses cell proliferation and bioenergetics. PMID: 24260536
    22. Data indicate that SDH5 is protected from mitochondrial LON protease (LONM)-mediated degradation in mitochondria by its stable interaction with SDHA, a state that is dysregulated in hereditary paraganglioma 2 (PGL2). PMID: 24414418
    23. Lon is overexpressed specifically in various types of cancer tissue including oral cancer. PMID: 23788038
    24. In cells with normal mitochondrial DNA levels, phosphorylated TFAM is degraded by Lon. PMID: 23201127
    25. Lon peptidase 1 (LONP1)-dependent breakdown of mitochondrial 5-aminolevulinic acid synthase protein by heme in human liver cells. PMID: 21659532
    26. Downregulation of mitochondrial lon protease impairs mitochondrial function and causes hepatic insulin resistance in human liver SK-HEP-1 cells. PMID: 21347624
    27. The promoter of Lon is at least part responsible for the upregulation of this protein during oxidative stress. PMID: 20933102
    28. Data show that Lon gene can be significantly downregulated by introduction of siRNA to result in enhanced sensitivity of MCF7 cells to UV, cisplatin and heat stress. PMID: 17584658
    29. may prevent extensive oxidation, aggregation and accumulation of aconitase, which could otherwise compromise mitochondrial function and cellular viability PMID: 12198491
    30. Lon participates directly in the metabolism of mtDNA. PMID: 14739292
    31. results indicate that mitochondrial Lon is required for normal survival and proliferation; a clear impetus for Lon's evolutionary conservation PMID: 15683722
    32. Results demonstrate that mitochondrial DNA binding is a physiological function of Lon and that cellular levels of Lon influence sensitivity to mtDNA damage. PMID: 17420247
    33. A review of the current knowledge on both catalytic mechanisms and inhibitors of Lon protease. PMID: 18021745
    34. Electrophoretic mobility shift assay and circular dichroism show that ssDNAs with a propensity for forming parallel G-quartets are specifically bound by hLon. PMID: 18174225

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  • 相关疾病：
    CODAS syndrome (CODASS)
  • 亚细胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Peptidase S16 family
  • 组织特异性：
    Duodenum, heart, lung and liver, but not thymus.
  • 数据库链接：

    HGNC: 9479

    OMIM: 600373

    KEGG: hsa:9361

    STRING: 9606.ENSP00000353826

    UniGene: Hs.350265