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. 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. Ubiquitous. 3 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: GEF; GEF, Ras; Mitochondrial
Chromosomal Location of Human Ortholog: 5|5 G2
Cellular Component:  membrane; mitochondrial inner membrane; mitochondrial membrane; mitochondrion
Molecular Function:  guanyl-nucleotide exchange factor activity; protein binding; Ran guanyl-nucleotide exchange factor activity; rRNA binding
Biological Process:  mitochondrial membrane fusion; positive regulation of mitochondrial translation
Reference #:  Q9CYF5 (UniProtKB)
Alt. Names/Synonyms: 5730496C04Rik; AU019812; OTTMUSP00000029415; RCC1-like G exchanging factor-like protein; Rcc1l; reculator of chromosome condensation 1 like; WBS16; Wbscr16; Williams-Beuren syndrome chromosomal region 16 protein homolog; Williams-Beuren syndrome chromosome region 16 homolog (human)
Gene Symbols: Rcc1l
Molecular weight: 49,995 Da
Basal Isoelectric point: 8.62  Predict pI for various phosphorylation states
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RCC1L

Protein Structure Not Found.


Cross-references to other databases:  STRING  |  BioGPS  |  Pfam  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene