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Leucine Rich PPR Motif Containing Protein (LRPPRC)

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LRP130; LSFC; GP130; 130 kDa leucine-rich protein; Leigh Syndrome,French-Canadian Type(Cytochrome Oxidase Deficiency)

Leucine Rich PPR Motif Containing Protein (LRPPRC)
CCC J931
HGNC 15714
MGI 1919666
PubMed 23878239
RGD 1306575
UniProt P42704
Wiki LRPPRC
LRPPRC contains 167 leucine residues that are dispersed every 15 to 19 amino acids throughout the protein, accounting for about 13% of the sequence. The N-terminal half of LRPPRC is cysteine rich.
LRPPRC has 4 potential N-linked glycosylation sites, a sequence that shares homology with the ATP-binding site of ATP-dependent kinases, and a protein kinase C phosphorylation site. It has no secretory signal sequence or hydrophobic domains. Northern blot analysis of HepG2 cells revealed expression of a major 4.8-kb transcript and minor transcripts of 5.2 and 7.0 kb. Western blot analysis of transiently transfected COS-1 cells revealed a doublet with an apparent molecular mass of about 130 kD.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Leucine Rich PPR Motif Containing Protein (LRPPRC) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Leucine Rich PPR Motif Containing Protein (LRPPRC) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Leucine Rich PPR Motif Containing Protein (LRPPRC) Polyclonal Antibody Customized Service Offer
Assay Kits SEJ931Hu ELISA Kit for Leucine Rich PPR Motif Containing Protein (LRPPRC) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Leucine Rich PPR Motif Containing Protein (LRPPRC) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Leucine Rich PPR Motif Containing Protein (LRPPRC) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Leucine Rich PPR Motif Containing Protein (LRPPRC) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Leucine Rich PPR Motif Containing Protein (LRPPRC) CLIA Kit Customized Service Offer
n/a ELISA Kit for Leucine Rich PPR Motif Containing Protein (LRPPRC) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Leucine Rich PPR Motif Containing Protein (LRPPRC) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Leucine Rich PPR Motif Containing Protein (LRPPRC) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Leucine Rich PPR Motif Containing Protein (LRPPRC) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Leucine Rich PPR Motif Containing Protein (LRPPRC) CLIA Kit Customized Service Offer
n/a ELISA Kit for Leucine Rich PPR Motif Containing Protein (LRPPRC) ELISA Kit Customized Service Offer
  1. "The role of the LRPPRC (leucine-rich pentatricopeptide repeat cassette) gene in cytochrome oxidase assembly: mutation causes lowered levels of COX (cytochrome c oxidase) I and COX III mRNA."Biochem. J. 382:331-336(2004) [PubMed] [Europe PMC] [Abstract]
  2. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  3. "Generation and annotation of the DNA sequences of human chromosomes 2 and 4." Nature 434:724-731(2005) [PubMed] [Europe PMC] [Abstract]
  4. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  5. "Molecular cloning and expression of the gene for a major leucine-rich protein from human hepatoblastoma cells (HepG2)."In Vitro Cell. Dev. Biol. Anim. 30A:111-114(1994) [PubMed] [Europe PMC] [Abstract]
  6. , Submitted (JUL-2007) to UniProtKB
  7. "Distinct RNP complexes of shuttling hnRNP proteins with pre-mRNA and mRNA: candidate intermediates in formation and export of mRNA."Mol. Cell. Biol. 21:7307-7319(2001) [PubMed] [Europe PMC] [Abstract]
  8. "Sequence analysis of LRPPRC and its SEC1 domain interaction partners suggests roles in cytoskeletal organization, vesicular trafficking, nucleocytosolic shuttling, and chromosome activity."Genomics 79:124-136(2002) [PubMed] [Europe PMC] [Abstract]
  9. "LRP130, a pentatricopeptide motif protein with a noncanonical RNA-binding domain, is bound in vivo to mitochondrial and nuclear RNAs."Mol. Cell. Biol. 23:4972-4982(2003) [PubMed] [Europe PMC] [Abstract]
  10. "LRP130, a single-stranded DNA/RNA-binding protein, localizes at the outer nuclear and endoplasmic reticulum membrane, and interacts with mRNA in vivo."Biochem. Biophys. Res. Commun. 317:736-743(2004) [PubMed] [Europe PMC] [Abstract]
  11. "New invMED1 element cis-activates human multidrug-related MDR1 and MVP genes, involving the LRP130 protein."Nucleic Acids Res. 32:3864-3876(2004) [PubMed] [Europe PMC] [Abstract]
  12. "Putative tumor suppressor RASSF1 interactive protein and cell death inducer C19ORF5 is a DNA binding protein."Biochem. Biophys. Res. Commun. 332:670-676(2005) [PubMed] [Europe PMC] [Abstract]
  13. "Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1alpha/LRP130 complex."Genes Dev. 20:2996-3009(2006) [PubMed] [Europe PMC] [Abstract]
  14. "Lysine acetylation targets protein complexes and co-regulates major cellular functions."Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
  15. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  16. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  17. "Identification of a gene causing human cytochrome c oxidase deficiency by integrative genomics."Proc. Natl. Acad. Sci. U.S.A. 100:605-610(2003) [PubMed] [Europe PMC] [Abstract]