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Immunogen sequence: TLLSSKTADV TKVWGMGLNK DSQLGFHRSR KDKTRGYEYV LEPSPVSLPL DRPQETRVLQ VSCGRAHSLV LTDREGVFSM GNNSYGQCGR KVVEN
Highest antigen sequence identity to the following orthologs: Mouse - 95%, Rat - 94%.
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: RCC1-like G exchanging factor-like protein; RCC1-like protein; Williams-Beuren syndrome chromosomal region 16 protein; Williams-Beuren syndrome chromosome region 16
Gene Aliases: RCC1L; WBSCR16
UniProt ID: (Human) Q96I51
Entrez Gene ID: (Human) 81554
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