| Function | 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. |
| Protein Name | Rcc1-Like G Exchanging Factor-Like ProteinRcc1-Like ProteinWilliams-Beuren Syndrome Chromosomal Region 16 Protein |
| Database Links | |
| Cellular Localisation | Mitochondrion MembraneIsoform 1: Mitochondrion Inner MembraneIsoform 2: Mitochondrion Inner MembraneIsoform 3: Mitochondrion Inner Membrane |
| Alternative Antibody Names | Anti-Rcc1-Like G Exchanging Factor-Like Protein antibodyAnti-Rcc1-Like Protein antibodyAnti-Williams-Beuren Syndrome Chromosomal Region 16 Protein antibodyAnti-RCC1L antibodyAnti-WBSCR16 antibody |
Information sourced from Uniprot.org
