Recombinant Human Leucine-rich PPR motif-containing protein, mitochondrial (LRPPRC), partial

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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HGNC: 15714

OMIM: 220111

KEGG: hsa:10128

STRING: 9606.ENSP00000260665

UniGene: Hs.368084