RCC1-like G exchanging factor-like protein (RCC1L)

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 (PubMed:28746876).

Plays an important 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 needed for intra-mitochondrial translation of core subunits of the oxidative phosphorylation system (PubMed:27667664).