Protein: 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)] (Q9CR68)
Organism: Mus musculus (Mouse)
Function: Component of the ubiquinol-cytochrome c oxidoreductase, a multisubunit transmembrane complex that is part of the mitochondrial electron transport chain which drives oxidative phosphorylation. 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 (By similarity).
Subcellular location: Mitochondrion inner membrane {ECO:0000269|PubMed:34616041, ECO:0000269|PubMed:38575788}; Single-pass membrane protein {ECO:0000269|PubMed:34616041, ECO:0000269|PubMed:38575788}.
Sequence: MLSVAARSGPFAPVLSATSRGVAGALRPLLQGAVPAASEPPVLDVKRPFLCRESLSGQAAARPLVATVGLNVPASVRFSHTDVKVPDFSDYRRAEVLDSTKSSKESSEARKGFSYLVTATTTVGVAYAAKNVVSQFVSSMSASADVLAMSKIEIKLSDIPEGKNMAFKWRGKPLFVRHRTKKEIDQEAAVEVSQLRDPQHDLDRVKKPEWVILIGVCTHLGCVPIANAGDFGGYYCPCHGSHYDASGRIRKGPAPLNLEVPAYEFTSDDVVVVG
InterPro domains: - IPR017941: Rieske [2Fe-2S] iron-sulphur domain (domain) [187-272]
- IPR037008: Cytochrome bc1 complex subunit Rieske, transmembrane domain superfamily (homologous_superfamily) [79-142]
- IPR005805: Rieske iron-sulphur protein, C-terminal (domain) [212-258]
- IPR004192: Cytochrome b-c1 complex subunit Rieske, transmembrane domain (domain) [80-145]
- IPR015248: Ubiquinol-cytochrome c reductase iron-sulphur subunit, N-terminal (domain) [2-77]
- IPR011070: Globular protein, non-globular alpha/beta subunit (homologous_superfamily) [1-57]
- IPR006317: Ubiquinol-cytochrome c reductase, iron-sulphur subunit (domain) [114-274]
- IPR014349: Rieske iron-sulphur protein (family) [79-274]
- IPR036922: Rieske [2Fe-2S] iron-sulphur domain superfamily (homologous_superfamily) [135-274]
Interaction partners: - Cytochrome c oxidase subunit 7C, mitochondrial
- Cytochrome b-c1 complex subunit 2, mitochondrial
- NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial
- Cytochrome c oxidase subunit 5A, mitochondrial
- Cytochrome b-c1 complex subunit 8
- Cytochrome c oxidase subunit 2
- Cytochrome c oxidase subunit 5B, mitochondrial
- NADH dehydrogenase iron-sulfur protein 4, mitochondrial
- Cytochrome b-c1 complex subunit 7
- NADH dehydrogenase flavoprotein 2, mitochondrial
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0003824 (catalytic activity), GO:0005215 (transporter activity), GO:0022857 (transmembrane transporter activity), GO:0016491 (oxidoreductase activity), GO:0009055 (electron transfer activity), GO:0016679 (oxidoreductase activity, acting on diphenols and related substances as donors), GO:0015075 (monoatomic ion transmembrane transporter activity), GO:0015318 (inorganic molecular entity transmembrane transporter activity), GO:0022804 (active transmembrane transporter activity), GO:0015399 (primary active transmembrane transporter activity), GO:0022890 (inorganic cation transmembrane transporter activity), GO:0022853 (active monoatomic ion transmembrane transporter activity), GO:0008324 (monoatomic cation transmembrane transporter activity), GO:0015453 (oxidoreduction-driven active transmembrane transporter activity), GO:0015078 (proton transmembrane transporter activity)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0009987 (cellular process), GO:0044237 (cellular metabolic process), GO:0006091 (generation of precursor metabolites and energy), GO:0015980 (energy derivation by oxidation of organic compounds), GO:0022900 (electron transport chain), GO:0045333 (cellular respiration), GO:0022904 (respiratory electron transport chain), GO:0019646 (aerobic electron transport chain), GO:0009060 (aerobic respiration), GO:0042773 (ATP synthesis coupled electron transport), GO:0042775 (mitochondrial ATP synthesis coupled electron transport), GO:0006119 (oxidative phosphorylation), GO:0006122 (mitochondrial electron transport, ubiquinol to cytochrome c)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005737 (cytoplasm), GO:0043229 (intracellular organelle), GO:0005739 (mitochondrion), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle)