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Proteins/UNIPROT:Q96I51
Guanine nucleotide exchange factor (GEF) for mitochondrial dynamin-related GTPase OPA1. Activates OPA1, by exchanging bound GDP for free GTP, and drives OPA1 and MFN1-dependent mitochondrial fusion. Plays an essential role in mitochondrial ribosome biogenesis. As a component of a functional protein-RNA module, consisting of RCC1L, NGRN, RPUSD3, RPUSD4, TRUB2, FASTKD2 and 16S mitochondrial ribosomal RNA (16S mt-rRNA), controls 16S mt-rRNA abundance and is required for intra-mitochondrial translation of core subunits of the oxidative phosphorylation system..
[Isoform 1]: Plays an essential role in mitochondrial ribosome biogenesis via its association with GTPases that play a role in the assembly of the large ribosome subunit..
[Isoform 2]: Plays an essential role in mitochondrial ribosome biogenesis via its association with GTPases that play a role in the assembly of the small ribosome subunit..
Function from UniProtKB (Q96I51), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
26 statements
26 statements · 2 reference databases · Subject: RCC1L · Sources: Hetionet, Reactome
3 names
Resolves to: RCC1L