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LRPPRC Antibody

  • 货号：
    CSB-PA013105GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    P42704
  • 基因名：
    LRPPRC
  • 别名：
    130 kDa leucine-rich protein antibody; gp130 antibody; Leucine-rich PPR motif-containing protein antibody; Leucine-rich PPR-motif containing protein antibody; LPPRC_HUMAN antibody; LRP 130 antibody; lrpprc antibody; mitochondrial antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human LRPPRC
  • 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 0.02% 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,IHC,IF
  • 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：
    May play a role in RNA metabolism in both nuclei and mitochondria. In the nucleus binds to HNRPA1-associated poly(A) mRNAs and is part of nmRNP complexes at late stages of mRNA maturation which are possibly associated with nuclear mRNA export. May bind mature mRNA in the nucleus outer membrane. In mitochondria binds to poly(A) mRNA. Plays a role in translation or stability of mitochondrially encoded cytochrome c oxidase (COX) subunits. May be involved in transcription regulation. Cooperates with PPARGC1A to regulate certain mitochondrially encoded genes and gluconeogenic genes and may regulate docking of PPARGC1A to transcription factors. Seems to be involved in the transcription regulation of the multidrug-related genes MDR1 and MVP. Part of a nuclear factor that binds to the invMED1 element of MDR1 and MVP gene promoters. Binds single-stranded DNA.
  • 基因功能参考文献：
    1. Study demonstrated that the LRPPRC-SLIRP complex is a global RNA chaperone that stabilizes RNA structures to expose the required sites for translation, stabilization, and polyadenylation. PMID: 29146908
    2. To investigate the impact of the OXPHOS defect in the liver, aothors analyzed the mitochondrial phenotype in mice harboring an hepatocyte-specific inactivation of Lrpprc. Loss of LRPPRC in the liver caused a generalized growth delay, and typical histological features of mitochondrial hepatopathy. PMID: 28575497
    3. There were no significant correlations between LRP130, SIRT3, or PGC-1alpha mRNA expression in response to acute sprint-interval training. Changes in protein expression of LRP130, SIRT3, and PGC-1alpha were positively correlated at several time points with large effect sizes, which suggest that the regulation of these proteins may be coordinated in human skeletal muscle. PMID: 27604398
    4. LRPPRC displays a broad and strong RNA binding capacity in vitro in contrast to SLIRP that associates only weakly with RNA. PMID: 27353330
    5. High expression of ULK1 concomitant with high expression of LRPPRC may serve as useful markers for shorter biochemical progression (BCP)-free survival and overall survival in patients with metastatic prostate cancer (PCa) after androgen deprivation therapy (ADT). PMID: 27679555
    6. This is the first study to report hypermethylation of LRPPRC, RAB6C, and ZNF471 in squamous cell carcinoma of the tongue PMID: 28255813
    7. LRPPRC knock-down in mammalian cells leads to an imbalance between mitochondria-encoded and nuclear-encoded subunits of complex IV PMID: 26412102
    8. study identifies LRPPRC as an important disease-causing gene in an early-onset, multisystem and neurological mitochondrial disease. PMID: 26510951
    9. LRPPRC levels were reduced in muscle cells and undetectable in liver from French Canadian Leigh Syndrome patients. PMID: 25214534
    10. LRPPRC is a transcription factor related to ABCB1 expression and highlight the importance of epigenetic regulation in CML resistance. PMID: 25089713
    11. Downregulation of LRPPRC expression resulted in the reduced expression of Bcl-2, upregulation of Bax, and cleaved caspase-9 and caspase-3. This induces apoptosis through the mitochondria-mediated pathway in PCa cells. PMID: 25379610
    12. LRPPRC functions as a checkpoint protein that prevents mitochondria from autophagy degradation and impact tumorigenesis. PMID: 24722279
    13. Tetherin binds with the mitochondrion-associated autophagy suppressor LRPPRC and prohibits its association with the autophagy initiation complex. PMID: 25631043
    14. LRPPRC overexpression is associated with gastric cancer. PMID: 24375316
    15. Data indicate that C14C10.4/MMA-1 Is the Structural and functional homolog of mammalian LRPPRC. PMID: 23878239
    16. LRPPRC therefore acts to suppress the initiation of basal levels of autophagy to clean up dysfunctional mitochondria and other cellular debris during the normal cell cycle. PMID: 23822101
    17. found that the tubulin-binding domain of NF1 is a binding partner of LRPPRC. Our findings provide clues to how loss or mutation of NF1 and LRPPRC may contribute to the manifestations of neurofibromatosis 1 and Leigh Syndrome, French Canadian variant. PMID: 23361976
    18. LRPPRC does not directly regulate mtDNA transcription but rather acts as a post-transcriptional regulator of mammalian mtDNA expression. PMID: 23599432
    19. These data identify LRPPRC as a HIV-1 factor that is involved in HIV-1 replication through more than one mechanism. PMID: 22808186
    20. The LRPPRC/SLIRP complex suppressed 3' exonucleolytic mRNA degradation mediated by PNPase and SUV3. PMID: 22661577
    21. LRP130 protein remodels mitochondria and stimulates fatty acid oxidation. PMID: 21971050
    22. LRP130 did not affect the capacity of hepatocarcinoma cells to extrude drugs since LRP130 down-regulation was insufficient to significantly reduce P-glycoprotein. PMID: 21109938
    23. Acute acidotic crises in a child with suspected mitochondrial disease may be suggestive of LRPPRC related COX deficiency. PMID: 21266382
    24. LRPPRC exists in a high-molecular-weight complex, and it coimmunoprecipitates with SLIRP, a stem-loop RNA-binding protein. PMID: 20200222
    25. LRPPRC protein is imported to the mitochondrial matrix and its mitochondrial targeting sequence is cleaved upon entry. PMID: 20633537
    26. Mitochondrial and nuclear genomic responses to loss of LRPPRC expression. PMID: 20220140
    27. regulatory role of LRPPRC in integration of cytoskeletal networks with vesicular trafficking, nucleocytosolic shuttling, transcription, chromosome remodeling, and cytokinesis PMID: 11827465
    28. using an integrative genomics approach, a single candidate gene, LRPPRC, was identified and shown to be the causative gene underlying Leigh syndrome, French-Canadian type (LSFC) PMID: 12529507
    29. The LRP130 protein has a role in transcription of the MDR1 and MVP genes. PMID: 15272088

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  • 相关疾病：
    Leigh syndrome French-Canadian type (LSFC)
  • 亚细胞定位：
    Mitochondrion. Nucleus, nucleoplasm. Nucleus inner membrane. Nucleus outer membrane. Note=Seems to be predominantly mitochondrial.
  • 组织特异性：
    Expressed ubiquitously. Expression is highest in heart, skeletal muscle, kidney and liver, intermediate in brain, non-mucosal colon, spleen and placenta, and lowest in small intestine, thymus, lung and peripheral blood leukocytes.
  • 数据库链接：

    HGNC: 15714

    OMIM: 220111

    KEGG: hsa:10128

    STRING: 9606.ENSP00000260665

    UniGene: Hs.368084