Cytochrome b-c1 complex subunit Rieske, mitochondrial (EC 7.1.1.8) (Complex III subunit 5) (Cytochrome b-c1 complex subunit 5) (Rieske iron-sulfur protein) (RISP) (Rieske protein UQCRFS1) (Ubiquinol-cytochrome c reductase iron-sulfur subunit) [Cleaved into: Cytochrome b-c1 complex subunit 9 (Su9) (Subunit 9) (8 kDa subunit 9) (Complex III subunit IX) (Cytochrome b-c1 complex subunit 11) (UQCRFS1 mitochondrial targeting sequence) (UQCRFS1 MTS) (Ubiquinol-cytochrome c reductase 8 kDa protein)]
1_MLSVA 6_ SRSGP 11_ FAPVL 16_ SATSR 21_ GVAGA 26_ LRPLV 31_ QATVP 36_ ATPEQ 41_ PVLDL 46_ KRPFL 51_ SRESL 56_ SGQAV 61_ RRPLV 66_ ASVGL 71_ NVPAS 76_ VCYSH 81_ TDIKV 86_ PDFSE 91_ YRRLE 96_ VLDST 101_ KSSRE 106_ SSEAR 111_ KGFSY 116_ LVTGV 121_ TTVGV 126_ AYAAK 131_ NAVTQ 136_ FVSSM 141_ SASAD 146_ VLALA 151_ KIEIK 156_ LSDIP 161_ EGKNM 166_ AFKWR 171_ GKPLF 176_ VRHRT 181_ QKEIE 186_ QEAAV 191_ ELSQL 196_ RDPQH 201_ DLDRV 206_ KKPEW 211_ VILIG 216_ VCTHL 221_ GCVPI 226_ ANAGD 231_ FGGYY 236_ CPCHG 241_ SHYDA 246_ SGRIR 251_ LGPAP 256_ LNLEV 261_ PTYEF 266_TSDDM
1: Component of the ubiquinol-cytochrome c oxidoreductase, a multisubunit transmembrane complex that is part of the mitochondrial electron transport chain which drives oxidative phosphorylation (PubMed:31883641). The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. The cytochrome b-c1 complex catalyzes electron transfer from ubiquinol to cytochrome c, linking this redox reaction to translocation of protons across the mitochondrial inner membrane, with protons being carried across the membrane as hydrogens on the quinol. In the process called Q cycle, 2 protons are consumed from the matrix, 4 protons are released into the intermembrane space and 2 electrons are passed to cytochrome c. The Rieske protein is a catalytic core subunit containing a [2Fe-2S] iron-sulfur cluster. It cycles between 2 conformational states during catalysis to transfer electrons from the quinol bound in the Q(0) site in cytochrome b to cytochrome c1 (By similarity). Incorporation of UQCRFS1 is the penultimate step in complex III assembly (PubMed:28673544)
2: Component of the ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII). UQCRFS1 undergoes proteolytic processing once it is incorporated in the complex III dimer. One of the fragments, called subunit 9, corresponds to its mitochondrial targeting sequence (MTS). The proteolytic processing is necessary for the correct insertion of UQCRFS1 in the complex III dimer, but the persistence of UQCRFS1-derived fragments may prevent newly imported UQCRFS1 to be processed and assembled into complex III and is detrimental for the complex III structure and function