RCC1-like G exchanging factor-like protein (RCC1-like) (Williams-Beuren syndrome chromosomal region 16 protein)
1_MALVA 6_ LVAGA 11_ RLGRR 16_ LSGPG 21_ LGRGH 26_ WTAAG 31_ RSRSR 36_ REAAE 41_ AEAEV 46_ PVVQY 51_ VGERA 56_ ARADR 61_ VFVWG 66_ FSFSG 71_ ALGVP 76_ SFVVP 81_ SSGPG 86_ PRAGA 91_ RPRRR 96_ IQPVP 101_ YRLEL 106_ DQKIS 111_ SAACG 116_ YGFTL 121_ LSSKT 126_ ADVTK 131_ VWGMG 136_ LNKDS 141_ QLGFH 146_ RSRKD 151_ KTRGY 156_ EYVLE 161_ PSPVS 166_ LPLDR 171_ PQETR 176_ VLQVS 181_ CGRAH 186_ SLVLT 191_ DREGV 196_ FSMGN 201_ NSYGQ 206_ CGRKV 211_ VENEI 216_ YSESH 221_ RVHRM 226_ QDFDG 231_ QVVQV 236_ ACGQD 241_ HSLFL 246_ TDKGE 251_ VYSCG 256_ WGADG 261_ QTGLG 266_ HYNIT 271_ SSPTK 276_ LGGDL 281_ AGVNV 286_ IQVAT 291_ YGDCC 296_ LAVSA 301_ DGGLF 306_ GWGNS 311_ EYLQL 316_ ASVTD 321_ STQVN 326_ VPRCL 331_ HFSGV 336_ GKVRQ 341_ AACGG 346_ TGCAV 351_ LNGEG 356_ HVFVW 361_ GYGIL 366_ GKGPN 371_ LVESA 376_ VPEMI 381_ PPTLF 386_ GLTEF 391_ NPEIQ 396_ VSRIR 401_ CGLSH 406_ FAALT 411_ NKGEL 416_ FVWGK 421_ NIRGC 426_ LGIGR 431_ LEDQY 436_ FPWRV 441_ TMPGE 446_ PVDVA 451_ CGVDH 456_MVTLA
1: 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 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 (PubMed:27667664)
2: 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
3: 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