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FLASH GENE
Symbol LRPPRC contributors: mct/pgu - updated : 21-12-2022
HGNC name leucine-rich PPR-motif containing
HGNC id 15714
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • N terminal multiple copies of leucine rich nuclear transport signal
  • ENTH, DUF28 and SEC1 homology domain
  • 20 PPR (pentatricopeptide repeats)
    • HOMOLOGY
    interspecies homolog to murine Lrpprc (75.4pc)
    homolog to rattus Lrpprc (76.4pc)
    Homologene
    FAMILY pentatricopeptide repeat (PPR) family
    CATEGORY RNA associated
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,matrix
    intracellular,cytoplasm,cytoskeleton,microtubule
    intracellular,nucleus,nucleoplasm
    intracellular,nuclear envelope,int
    intracellular,nuclear envelope,ext
    text
  • inner and outer membrane of nucleus
  • predominantly mitochondrial
  • primarily a mitochondrial protein, localized to the mitochondrial matrix
  • core mitochondrial nucleiod protein
    • basic FUNCTION
  • RNA-binding protein that is a constituent of postsplicing nuclear RNP complexes associated with mature mRNA
  • multidomain organizer that potentially integrates mitochondria and the microtubular cytoskeleton with chromosome remodeling
  • involved in integration of cytoskeletal networks with vesicular trafficking, nucleocytosolic shutting, chromosome remodeling
  • participating in mRNA processing both in the nucleus and the mitochondrion
  • having a role in transcription of the ABCB1 and MVP genes
  • may function as a chaperone involved in the assembly of mitochondrial complex IV, although it also binds nucleic acids
  • required for the nuclear export arm of the regulon may function as energy sensors in the cell, coupling proliferation and survival to energy status
  • involved in the expression of genes related to hexose metabolism, prostaglandin synthesis and glycosphingolipid biology that may either play an adaptive role in cell survival or contribute to LSFC pathogenesis
  • plays a role in translation or stability of mitochondrially encoded cytochrome c oxidase subunits
  • activates mitochondrial transcription, activation that is associated with remodeling of mitochondria
  • stimulates oxidative phosphorylation
  • stimulates OXPHOS by promoting supercomplex formation, which increases cristae density
  • transcriptional activator of mitochondrial transcription
  • LRPPRC forms an RNA-dependent protein complex that is necessary for maintaining a pool of non-translated mRNAs in mammalian mitochondria
  • acts at the post-transcriptional level to stabilize mitochondrial mRNAs, to promote mitochondrial mRNA polyadenylation, and to coordinate mitochondrial translation
  • LRPPRC does not directly regulate mtDNA transcription but rather acts as a post-transcriptional regulator of mammalian mtDNA expression
  • LRPPRC functions as a checkpoint protein that prevents mitochondria from autophagy degradation and impact tumorigenesis
  • multi-functional protein, that regulates a myriad of biological processes, including energy metabolism and maturation and the export of nuclear mRNA
  • LRPPRC acted as an inhibitor of autophagy in human cancer cells
  • LRPPRC may act as an oncogene via maintaining mitochondrial homeostasis
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • part of nmRNP complexes at late stages of mRNA maturation which are possibly associated with nuclear mRNA export
  • LRPPRC-SLIRP complex is a global RNA chaperone that stabilizes RNA structures to expose the required sites for translation, stabilization, and polyadenylation
    • INTERACTION
    DNA
  • binding single stranded DNA
    • RNA
  • binding to HNRPA1-associated mRNAs in the nucleus
  • may bind mature RNA in the nucleus outer membrane
  • binding poly(A)mRNA int eh mitochondria
    • small molecule
    protein
  • interacting with C6orf34, CECR2
  • interaction with UXT (increasing concentrations of UXT contributes to progressive aggregation of mitochondria and cell death potentially through association of UXT with LRPPRC)
  • interaction with EIF4E (promotes mRNA export by recruiting EIF4E away from its potent inhibitor, PML )
  • interacting with HEPB2, MAP1S, C19orf2, PPARGC1A, FOXO1
  • cooperating with PPARGC1A to regulate certain mitochondrially encoded genes and gluconeogenic genes and may regulate docking of PPARGC1A tot ranscription factors
  • MAP1S interacted with mitochondrion-associated leucine-rich PPR-motif containing protein (LRPPRC) that interacts with the mitophagy initiator and Parkinson disease-related protein Parkin
  • increases gene expression of mitochondrially encoded genes, 13 of which encode OXPHOS subunits
  • LRPPRC/SLIRP suppresses PNPase-mediated mRNA decay and promotes polyadenylation in mitochondria
  • LRPPRC interacts with MAP1S that interacts with MAP1LC3A and bridges autophagy components with microtubules and mitochondria to affect autophagy flux
  • distinct functions for SLIRP and LRPPRC within the LRPPRC-SLIRP complex, with a novel role for SLIRP in mitochondrial translation
  • despite the prediction that specific amino acids in LRPPRC and SLIRP should bind RNA, they are instead used to facilitate protein-protein interactions, enabling the formation of a stable complex between these two proteins
  • LRPPRC interacts with MAP1S that promotes autophagy initiation and maturation to suppress genomic instability and tumorigenesis
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) LSFC
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    in gastric cancer and higher LRPPRC expression showed a poorer overall survival rate than those with lower LRPPRC expression
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
  • could be used as a predictive marker for patient prognosis of gastric cancer
  • could be used as a predictive marker for patient prognosis in pancreatic cancer
    • Therapy target
    SystemTypeDisorderPubmed
    cancerdigestivestomach
    may be a novel therapeutic target for gastric cancer
    ANIMAL & CELL MODELS