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ABCE1

Function

Cotranslational quality control factor involved in the No-Go Decay (NGD) pathway (PubMed:21448132). Together with PELO and HBS1L, is required for 48S complex formation from 80S ribosomes and dissociation of vacant 80S ribosomes (PubMed:21448132). Together with PELO and HBS1L, recognizes stalled ribosomes and promotes dissociation of elongation complexes assembled on non-stop mRNAs; this triggers endonucleolytic cleavage of the mRNA, a mechanism to release non-functional ribosomes and to degrade damaged mRNAs as part of the No-Go Decay (NGD) pathway (PubMed:21448132). Plays a role in the regulation of mRNA turnover (By similarity). Plays a role in quality control of translation of mitochondrial outer membrane-localized mRNA (PubMed:29861391). As part of the PINK1-regulated signaling, ubiquitinated by CNOT4 upon mitochondria damage; this modification generates polyubiquitin signals that recruit autophagy receptors to the mitochondrial outer membrane and initiate mitophagy (PubMed:29861391). RNASEL-specific protein inhibitor which antagonizes the binding of 2-5A (5'-phosphorylated 2',5'-linked oligoadenylates) to RNASEL (PubMed:9660177). Negative regulator of the anti-viral effect of the interferon-regulated 2-5A/RNASEL pathway (PubMed:9660177, PubMed:9847332, PubMed:11585831).

(Microbial infection) May act as a chaperone for post-translational events during HIV-1 capsid assembly.

(Microbial infection) Plays a role in the down-regulation of the 2-5A/RNASEL pathway during encephalomyocarditis virus (EMCV) and HIV-1 infections.

Post-translational modifications

Ubiquitinated by CNOT4 (PubMed:29861391). Ubiquitination mediates the recruitment of autophagy receptors to the mitochondrial outer membrane and initiates mitophagy (PubMed:29861391).

Sequence similarities

Belongs to the ABC transporter superfamily. ABCE family.

Cellular localization

  • Cytoplasm
  • Mitochondrion
  • Localized to clusters of virus formation at the plasma membrane.

Alternative names

  • ATP-binding cassette sub-family E member 1
  • 2'-5'-oligoadenylate-binding protein
  • HuHP68
  • RNase L inhibitor
  • Ribonuclease 4 inhibitor
  • RNS4I
  • ABCE1
  • RLI
  • RNS4I
  • RNASEL1
  • OK/SW-cl.40
  • RNASELI

Target type

Proteins

Molecular weight

67314Da