LRPPRC Antibody

Code CSB-PA013105LA01HU
Size US$166
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Image
  • Western Blot
    Positive WB detected in: Hela whole cell lysate, 293T whole cell lysate, HepG2 whole cell lysate
    All lanes: LRPPRC antibody at 4µg/ml
    Secondary
    Goat polyclonal to rabbit IgG at 1/50000 dilution
    Predicted band size: 158 kDa
    Observed band size: 158 kDa

  • IHC image of CSB-PA013105LA01HU diluted at 1:400 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

  • IHC image of CSB-PA013105LA01HU diluted at 1:400 and staining in paraffin-embedded human spleen tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

  • Immunofluorescence staining of HepG2 cells with CSB-PA013105LA01HU at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).

  • Immunoprecipitating LRPPRC in HepG2 whole cell lysate
    Lane 1: Rabbit control IgG instead of CSB-PA013105LA01HU in HepG2 whole cell lysate. For western blotting, a HRP-conjugated Protein G antibody was used as the secondary antibody (1/2000)
    Lane 2: CSB-PA013105LA01HU (6µg) + HepG2 whole cell lysate (500µg)
    Lane 3: HepG2 whole cell lysate (20µg)

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) LRPPRC Polyclonal antibody
Uniprot No.
Target Names
LRPPRC
Alternative Names
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
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Leucine-rich PPR motif-containing protein, mitochondrial protein (901-1036AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The LRPPRC Antibody (Product code: CSB-PA013105LA01HU) is Non-conjugated. For LRPPRC Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA013105LB01HU LRPPRC Antibody, HRP conjugated ELISA
FITC CSB-PA013105LC01HU LRPPRC Antibody, FITC conjugated
Biotin CSB-PA013105LD01HU LRPPRC Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA, WB, IHC, IF, IP
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:5000
IHC 1:200-1:500
IF 1:50-1:200
IP 1:200-1:2000
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
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.

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Target Background

Function
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.
Gene References into Functions
  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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Involvement in disease
Leigh syndrome French-Canadian type (LSFC)
Subcellular Location
Mitochondrion. Nucleus, nucleoplasm. Nucleus inner membrane. Nucleus outer membrane. Note=Seems to be predominantly mitochondrial.
Tissue Specificity
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.
Database Links

HGNC: 15714

OMIM: 220111

KEGG: hsa:10128

STRING: 9606.ENSP00000260665

UniGene: Hs.368084

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