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Respiratory electron transport
Transfer of electrons through mitochondrial respiratory complexes and mobile carriers. 47 reactions, from Reactome R-HSA-611105.
- Transfer of electrons through the succinate dehydrogenase complexCoQ + SUCCA → FUMA + CoQH2
- Electron transfer from reduced cytochrome c to molecular oxygenO2 + 12 H+ + 4 Cytochrome c (reduced) → 2 H2O + 8 H+ + 4 Cytochrome c (oxidised)
- Complex I oxidises NADH to NAD+, reduces CoQ to CoQH2NADH + 5 H+ + CoQ → NAD+ + 4 H+ + CoQH2
- Electron transfer from ubiquinol to cytochrome c of complex IIICoQ + 2 H+ + 2 Cytochrome c (oxidised) + 2 CoQH2 → 2 CoQ + CoQH2 + 4 H+ + 2 Cytochrome c (reduced)
- Reducing equivalents from beta-oxidation of fatty acids transfer to ETFFADH2 + ETF:FAD → ETF:FADH2 + FAD
- ETFDH oxidises ETF (reduced) to ETF, reduces CoQ to CoQH2ETF:FADH2 + CoQ → ETF:FAD + H+ + CoQH2
- SLC25A12,13 exchange L-Glu and L-AspL-Asp + L-Glu + H+ → L-Asp + L-Glu + H+
- SLC25A11 exchanges 2-OG and MALMAL + 2OG → MAL + 2OG
- NUBPL binds 4Fe-4SNUBPL + 4Fe-4S → NUBPL:4Fe-4S
- NUBPL transfers 4Fe-4S to Complex I subunitsNDUF subunits + NUBPL:4Fe-4S → NDUF:4Fe-4S subunits + NUBPL
- NUBPL transfers 4Fe-4S to NDUFV1, V2NDUFV1 + 2 NUBPL:4Fe-4S + NDUFV2 + FMN → NDUFV1:4Fe-4S:FMN + NDUFV2:4Fe-4S + 2 NUBPL
- Intermediate 1 binds HP subcomplex to form Intermediate 2HP subcomplex + Intermediate 1 → Intermediate 2
- Peripheral arm subunits bind the 815kDa complex to form a 980kDa complex815kDa complex + FP subcomplex + NDUFS5 → 980kDa complex
- Intermediate 2 binds MT-ND1:NDUFAF5:NDUFAF6 to form a 315kDa subcomplexIntermediate 2 + MT-ND1 + NDUFAF5 + NDUFAF6 → 315kDa subcomplex
- The MCIA complex, NDUFAF2-7 all dissociate from the 980kDa complex, resulting in Complex I980kDa complex → Complex I + MCIA complex + NDUFAF2 + NDUFAF3 + NDUFAF4 + NDUFAF5 + NDUFAF6 + NDUFAF7 + TIMMDC1
- ND4, ND5 bind the 550kDa complex to form the 815kDa complex550kDa complex + MT-ND4 + MT-ND5 → 815kDa complex
- COA1:MT-ND2, TMEM186:MT-ND3, MT-ND6, NDUFB6 bind the MCIA complex to form a 370kDa subcomplexMCIA complex + MT-ND6 + NDUFB6 + COA1:MT-ND2 + TMEM186:MT-ND3 → 370 kDa subcomplex
- The 315kDa subcomplex binds the 370kDa subcomplex to form the 550kDa complex315kDa subcomplex + 370 kDa subcomplex → 550kDa complex
- IP subcomplex binds NDUFAF3, NDUFAF4, TIMMDC1 to form Intermediate 1IP subcomplex + NDUFAF3 + NDUFAF4 + TIMMDC1 → Intermediate 1
- NDUF subunits bind to form the IP subcomplexNDUFA9:FAD + NDUFAF7:NDUFS2:2x4Fe-4S + NDUFS3 + NDUFS7:4Fe-4S + NDUFS8:2x4Fe-4S → IP subcomplex
- NDUF subunits bind to form the FP subcomplexNDUFA12 + NDUFAF2 + NDUFS1:2x4Fe-4S + NDUFS4 + NDUFS6 + NDUFV1:4Fe-4S:FMN + NDUFV2:4Fe-4S + NDUFV3 → FP subcomplex
- PXLP-K259-GOT1 transaminates 2-OG and L-AspL-Asp + 2OG → OA + L-Glu
- GOT2 transaminates OA and L-GluOA + L-Glu → L-Asp + 2OG
- MDH2 dimer dehydrogenates MALMAL + NAD+ → OA + NADH + H+
- SLC25A18,22 import L-Glu, H+L-Glu + H+ → L-Glu + H+
- MDH1 reduces OANADH + OA + H+ → MAL + NAD+
- MT-CO1 mRNA is translatedMT-CO1 mRNA → nascent MT-CO1
- Nascent MT-CO1 binds to MITRAC, Mg2+, PE, CLMITRAC + nascent MT-CO1 + Mg2+ + PE + cardiolipin → MT-CO1:Mg2+:PE:CL:MITRAC
- TIMM21 carries COX4, COX5A, COX6C to MT-CO1:MITRACTIMM21:COX4:COX5A:COX6C + MT-CO1:Mg2+:PE:CL:MITRAC → MT-CO1:Mg2+:PE:CL:MITRAC:TIMM21:COX4:COX5A:COX6C
- Metallochaperone inserts Cu2+ into MT-CO1MT-CO1:Mg2+:PE:CL:MITRAC:TIMM21:COX4:COX5A:COX6C + COX17:Cu → MT-CO1:Cu2+:Mg2+:PE:CL:MITRAC:TIMM21:COX4:COX5A:COX6C + COX17
- MT-CO2 mRNA is translatedMT-CO2 mRNA → nascent MT-CO2
- MT-CO1 and MT-CO2 complexes associate, installing heme moietiesCOX7B + COX7C + MT-CO1:Cu2+:Mg2+:PE:CL:MITRAC:TIMM21:COX4:COX5A:COX6C + MT-CO2:Mg2+:CuA + 3 heme A + COX8A,C → holo-MT-CO1,2 complex + TIMM21
- Metallochaperone inserts 2Cu2+ into MT-CO22 COX17:Cu + MT-CO2:COX20:TMEM177 → 2 COX17 + MT-CO2:Mg2+:CuA + COX20:TMEM177
- MT-CO3, COX6A,B,7A and NDUFA4 bind to holo-MT-CO1,2 complexMT-CO3:PE:CL + NDUFA4:CL + COX7A1,2,2L + COX6A:PE + COX6B1,2 + holo-MT-CO1,2 complex → Cytochrome c oxidase + MITRAC
- MT-CYB is translatedMT-CYB mRNA + UQCC1:UQCC2:UQCC5:UQCC6 → Intermediate 0
- Intermediate 0 binds heme and UQCC3UQCC3 + heme b + Intermediate 0 → Intermediate I
- Intermediate I binds second heme, UQCRB, UQCRQUQCRB + UQCRQ + Intermediate I + heme b → Intermediate II + UQCC1:UQCC2:UQCC5:UQCC6
- Intermediate II binds CYC1, UQCRC1, UQCRC2, UQCRHCYC1:heme c + Intermediate II + UQCRC1 + UQCRC2 + UQCRH, (UQCRHL) → Late core complex monomer + UQCC3
- Late core complex dimerizes2 Late core complex monomer → Late core complex dimer
- Late core complex dimer binds UQCR10, UQCR11, UQCRFS1.2 UQCR10 + 2 UQCR11 + Late core complex dimer + 2 holo-UQCRFS1(1-274) → Dimer of Late core complex:UQCR10:UQCR11:holo-UQCRFS1(1-274)
- HCCS inserts heme into cytochrome cheme b + apo-CYCS → Cytochrome c (reduced)
- apo-UQCRFS1 binds LYRM7LYRM7 + UQCRFS1(1-274) → UQCRFS1(1-274):LYRM7
- 2Fe-2S is inserted in UQCRFS1FXN:NFS1:ISD11:ISCU:2Fe-2S Cluster + UQCRFS1(1-274):LYRM7 → FXN:ISD11:NFS1:ISCU + holo-UQCRFS1(1-274) + LYRM7
- COX18 inserts nascent MT-CO2 in COX20:TMEM77Mg2+ + COX20:TMEM177 + nascent MT-CO2 → MT-CO2:COX20:TMEM177
- Unknown peptidase cleaves UQCRFS1 subunitDimer of Late core complex:UQCR10:UQCR11:holo-UQCRFS1(1-274) → Dimer of Late core complex:UQCR10:UQCR11:holo-UQCRFS1(79-274):Fragments
- TTC19 clears UQCRFS1 fragments from Complex IIIDimer of Late core complex:UQCR10:UQCR11:holo-UQCRFS1(79-274):Fragments → Cytochrome c reductase + 2 Unspecified N-terminal UQCRFS1 fragments
- MT-ND4 is translatedMT-ND4 mRNA → MT-ND4