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FLASH GENE
Symbol SLIRP contributors: mct/ - updated : 27-12-2022
HGNC name chromosome 14 open reading frame 156
HGNC id 20495
Corresponding disease
Mt-ECMOD mitochondrial encephalomyopathy with OXPHOS deficiency
Location 14q24.3      Physical location : -
Genatlas name SRA stem-loop-interacting RNA-binding protein, mitochondrial
Synonym symbol(s) DC23, DC50, PD04872, C14orf156
DNA
TYPE functioning gene
STRUCTURE 9.53 kb     4 Exon(s)
MAPPING cloned Y linked N status provisional
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
4 - 436 12 109 - 2020 33150185
4 - 436 - 92 - 2020 33150185
4 - 414 - 107 - 2020 33150185
EXPRESSION
Type widely
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Digestiveliver   highly Homo sapiens
 stomach   highly
Nervousbrain   highly Homo sapiens
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Blood / hematopoieticbone marrow  highly
Muscularstriatumskeletal highly Homo sapiens
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • two ubiquitination sites (Lys36, Lys88) within an RNA recognition motif domain (Ala21-Val91), which is essential for correct association of mt-mRNAs with the mitoribosome (
  • HOMOLOGY
    Homologene
    FAMILY
    CATEGORY regulatory
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria
    intracellular,nucleus
    intracellular,nuclear envelope
    basic FUNCTION
  • modulates modulates nuclear receptors transactivation and may regulate mitochondrial function
  • plays an essential role in maintaining normal steady-state levels of all mt-mRNA transcripts encoding structural OXPHOS proteins
  • involved in regulating male fertility, wherein its loss results in asthenozoospermia associated with compromised sperm structure and mitochondrial morphology
  • SLIRP regulates male fertility, and SLIRP and sperm progressive motility are positively correlated
  • stem-loop RNA-binding protein that regulates mitochondrial RNA expression and oxidative phosphorylation (OXPHOS)
  • may play a role in the pathophysiology of mitochondrial diseases
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • LRPPRC-SLIRP complex is a global RNA chaperone that stabilizes RNA structures to expose the required sites for translation, stabilization, and polyadenylation
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • colocalizes with SNW1, another nuclear receptor (NR) coregulator, and reduces SNW1-potentiated NR signaling
  • interacting with NCOA1, NCOR
  • LRPPRC/SLIRP suppresses PNPase-mediated mRNA decay and promotes polyadenylation in mitochondria
  • SLIRP is a N42-binding protein which may regulate DAZAP1 subcellular localization
  • 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
  • can interact directly with guanine quadruplex (G4) DNA, which underscored the complex regulatory networks involved in G4 biology
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) Mt-ECMOD
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    and oxidative damage is increased, with energy metabolism decreased in spermatozoa of asthenospermia patients compared to normospermia
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • Slirp KO mice produced significantly fewer progressively motile sperm than WT animals