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8UCA

Formation of I2+III2 supercomplex rescues respiratory chain defects

This is a non-PDB format compatible entry.
Entity
Entity IDChain IDDescriptionTypeChain lengthFormula weightNumber of moleculesDB Name (Accession)Biological sourceDescriptive keywords
1A, OA
(3, 3a)
NADH-ubiquinone oxidoreductase chain 3polymer11513223.82UniProt (P03899)
Pfam (PF00507)
Mus musculus (house mouse)
2B, PA
(S3, s3)
NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrialpolymer26330191.32UniProt (Q9DCT2)
Pfam (PF00329)
Mus musculus (house mouse)Complex I-30kD,CI-30kD,NADH-ubiquinone oxidoreductase 30 kDa subunit
3C, QA
(S2, s2)
NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrialpolymer46352720.62UniProt (Q91WD5)
Pfam (PF00346)
Mus musculus (house mouse)Complex I-49kD,CI-49kD,NADH-ubiquinone oxidoreductase 49 kDa subunit
4D, RA
(1, 1a)
NADH-ubiquinone oxidoreductase chain 1polymer31836105.02UniProt (Q4JFN6)
Pfam (PF00146)
Mus musculus (house mouse)
5E, SA
(6, 6a)
NADH-ubiquinone oxidoreductase chain 6polymer17218628.12UniProt (P03925)
Pfam (PF00499)
Mus musculus (house mouse)NADH dehydrogenase subunit 6
6F, TA
(4L, 4l)
NADH-ubiquinone oxidoreductase chain 4Lpolymer9810609.62UniProt (P03903)
Pfam (PF00420)
Mus musculus (house mouse)NADH dehydrogenase subunit 4L
7G, UA
(5, 5a)
NADH-ubiquinone oxidoreductase chain 5polymer60768519.32UniProt (P03921)
Pfam (PF00662)
Pfam (PF00361)
Pfam (PF06455)
Mus musculus (house mouse)NADH dehydrogenase subunit 5
8H, VA
(4, 4a)
NADH-ubiquinone oxidoreductase chain 4polymer45951915.52UniProt (P03911)
Pfam (PF01059)
Pfam (PF00361)
Mus musculus (house mouse)NADH dehydrogenase subunit 4
9I, WA
(2, 2a)
NADH-ubiquinone oxidoreductase chain 2polymer34538772.22UniProt (P03893)
Pfam (PF00361)
Pfam (PF06444)
Mus musculus (house mouse)NADH dehydrogenase subunit 2
10J, XA
(AL, al)
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrialpolymer35540657.42UniProt (Q99LC3)
Pfam (PF01712)
Mus musculus (house mouse)Complex I-42kD,CI-42kD,NADH-ubiquinone oxidoreductase 42 kDa subunit
11K, YA
(A9, a9)
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrialpolymer37742588.12UniProt (Q9DC69)
Pfam (PF05368)
Mus musculus (house mouse)
12L, ZA
(S4, s4)
NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrialpolymer17519814.72UniProt (Q9CXZ1)
Pfam (PF04800)
Mus musculus (house mouse)Complex I-18 kDa,CI-18 kDa,Complex I-AQDQ,CI-AQDQ,NADH-ubiquinone oxidoreductase 18 kDa subunit
13AB, M
(S6, s6)
NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrialpolymer11613041.82UniProt (P52503)
Pfam (PF10276)
Mus musculus (house mouse)Complex I-13kD-A,CI-13kD-A,NADH-ubiquinone oxidoreductase 13 kDa-A subunit
14BB, N
(A2, a2)
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2polymer9910932.72UniProt (Q9CQ75)
Pfam (PF05047)
Mus musculus (house mouse)Complex I-B8,CI-B8,NADH-ubiquinone oxidoreductase B8 subunit
15CB, DB, O, P
(AB, AC, ab, ac)
Acyl carrier protein, mitochondrialpolymer15617390.34UniProt (Q9CR21)
Pfam (PF00550)
Mus musculus (house mouse)ACP,CI-SDAP,NADH-ubiquinone oxidoreductase 9.6 kDa subunit
16EB, Q
(A5, a5)
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5polymer11613380.72UniProt (Q9CPP6)
Pfam (PF04716)
Mus musculus (house mouse)Complex I subunit B13,Complex I-13kD-B,CI-13kD-B,NADH-ubiquinone oxidoreductase 13 kDa-B subunit
17FB, R
(A6, a6)
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6polymer13115311.92UniProt (Q9CQZ5)
Pfam (PF13233)
Mus musculus (house mouse)Complex I-B14,CI-B14,NADH-ubiquinone oxidoreductase B14 subunit
18GB, S
(A8, a8)
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8polymer17220025.12UniProt (Q9DCJ5)
Pfam (PF06747)
Mus musculus (house mouse)Complex I-19kD,CI-19kD,Complex I-PGIV,CI-PGIV,NADH-ubiquinone oxidoreductase 19 kDa subunit
19HB, T
(AM, am)
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11polymer14214999.22UniProt (G5E814)
Pfam (PF02466)
Mus musculus (house mouse)
20IB, U
(AO, ao)
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13polymer14416881.62UniProt (Q9ERS2)
Pfam (PF06212)
Mus musculus (house mouse)Cell death regulatory protein GRIM-19,Complex I-B16.6,CI-B16.6,Gene associated with retinoic and interferon-induced mortality 19 protein,GRIM-19,Gene associated with retinoic and IFN-induced mortality 19 protein,NADH-ubiquinone oxidoreductase B16.6 subunit
21JB, V
(A1, a1)
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1polymer708149.52UniProt (O35683)
Pfam (PF15879)
Mus musculus (house mouse)Complex I-MWFE,CI-MWFE,NADH-ubiquinone oxidoreductase MWFE subunit
22KB, W
(A3, a3)
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3polymer849338.92UniProt (Q9CQ91)
Pfam (PF14987)
Mus musculus (house mouse)Complex I-B9,CI-B9,NADH-ubiquinone oxidoreductase B9 subunit
23LB, X
(C1, c1)
NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrialpolymer768636.02UniProt (Q9CQY9)
Pfam (PF15088)
Mus musculus (house mouse)Complex I-KFYI,CI-KFYI,NADH-ubiquinone oxidoreductase KFYI subunit
24MB, Y
(C2, c2)
NADH dehydrogenase [ubiquinone] 1 subunit C2polymer12014185.72UniProt (Q9CQ54)
Pfam (PF06374)
Mus musculus (house mouse)
25NB, Z
(S5, s5)
NADH dehydrogenase [ubiquinone] iron-sulfur protein 5polymer10612675.82UniProt (Q99LY9)
Pfam (PF10200)
Mus musculus (house mouse)Complex I-15 kDa,CI-15 kDa,NADH-ubiquinone oxidoreductase 15 kDa subunit
26AA, OB
(B1, b1)
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1polymer576965.12UniProt (P0DN34)
Pfam (PF08040)
Mus musculus (house mouse)Complex I-MNLL,CI-MNLL,NADH-ubiquinone oxidoreductase MNLL subunit
27BA, PB
(BM, bm)
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrialpolymer15117463.72UniProt (O09111)
Pfam (PF10183)
Mus musculus (house mouse)Complex I-ESSS,CI-ESSS,NADH-ubiquinone oxidoreductase ESSS subunit,Neuronal protein 15.6,Np15.6,p15.6
28CA, QB
(B5, b5)
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrialpolymer18921742.22UniProt (Q9CQH3)
Pfam (PF09781)
Mus musculus (house mouse)Complex I-SGDH,CI-SGDH,NADH-ubiquinone oxidoreductase SGDH subunit
29DA, RB
(B6, b6)
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6polymer12715408.92UniProt (Q3UIU2)
Pfam (PF09782)
Mus musculus (house mouse)Complex I-B17,CI-B17,NADH-ubiquinone oxidoreductase B17 subunit
30EA, SB
(B2, b2)
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrialpolymer10511982.42UniProt (Q9CPU2)
Pfam (PF14813)
Mus musculus (house mouse)Complex I-AGGG,CI-AGGG,NADH-ubiquinone oxidoreductase AGGG subunit
31FA, TB
(B3, b3)
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3polymer10411714.22UniProt (Q9CQZ6)
Pfam (PF08122)
Mus musculus (house mouse)Complex I-B12,CI-B12,NADH-ubiquinone oxidoreductase B12 subunit
32GA, UB
(B8, b8)
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrialpolymer18621903.82UniProt (Q9D6J5)
Pfam (PF05821)
Mus musculus (house mouse)Complex I-ASHI,CI-ASHI,NADH-ubiquinone oxidoreductase ASHI subunit
33HA, VB
(B4, b4)
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4polymer12915105.32UniProt (Q9CQC7)
Pfam (PF07225)
Mus musculus (house mouse)Complex I-B15,CI-B15,NADH-ubiquinone oxidoreductase B15 subunit
34IA, WB
(B9, b9)
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9polymer17922020.12UniProt (Q9CQJ8)
Pfam (PF05347)
Mus musculus (house mouse)Complex I-B22,CI-B22,NADH-ubiquinone oxidoreductase B22 subunit
35JA, XB
(B7, b7)
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7polymer13616229.62UniProt (Q9CR61)
Pfam (PF05676)
Mus musculus (house mouse)Complex I-B18,CI-B18,NADH-ubiquinone oxidoreductase B18 subunit
36KA, YB
(BL, bl)
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10polymer17621054.82UniProt (Q9DCS9)
Pfam (PF10249)
Mus musculus (house mouse)Complex I-PDSW,CI-PDSW,NADH-ubiquinone oxidoreductase PDSW subunit
37LA, ZB
(AN, an)
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12polymer14517112.42UniProt (Q7TMF3)
Pfam (PF05071)
Mus musculus (house mouse)Complex I-B17.2,CI-B17.2,CIB17.2,NADH-ubiquinone oxidoreductase subunit B17.2
38AC, MA
(A7, a7)
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7polymer11212464.42UniProt (Q9Z1P6)
Pfam (PF07347)
Mus musculus (house mouse)Complex I-B14.5a,CI-B14.5a,NADH-ubiquinone oxidoreductase subunit B14.5a
39BC, NA
(V3, v3)
NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrialpolymer10411833.52UniProt (Q8BK30)
Pfam (PF15880)
Mus musculus (house mouse)Complex I-9kD,CI-9kD,NADH-ubiquinone oxidoreductase 9 kDa subunit
40CC, MC
(3A, 3L)
Cytochrome b-c1 complex subunit 1, mitochondrialpolymer44649355.32UniProt (Q9CZ13)
Pfam (PF00675)
Pfam (PF05193)
Mus musculus (house mouse)
41DC, NC
(3B, 3M)
Cytochrome b-c1 complex subunit 2, mitochondrialpolymer43946641.82UniProt (Q9DB77)
Pfam (PF00675)
Pfam (PF05193)
Mus musculus (house mouse)
42EC, OC
(3C, 3N)
Cytochrome bpolymer38043143.22UniProt (P00158)
Pfam (PF00032)
Pfam (PF00033)
Mus musculus (house mouse)
43FC, PC
(3D, 3O)
Cytochrome c1, heme protein, mitochondrialpolymer24127312.22UniProt (Q9D0M3)
Pfam (PF02167)
Mus musculus (house mouse)
44GC, QC
(3E, 3P)
Cytochrome b-c1 complex subunit 9polymer19621524.42UniProt (Q9CR68)
Pfam (PF02921)
Pfam (PF00355)
Mus musculus (house mouse)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
45HC, RC
(3F, 3Q)
Cytochrome b-c1 complex subunit 7polymer11013456.32UniProt (Q9CQB4)
Pfam (PF02271)
Mus musculus (house mouse)
46IC, SC
(3G, 3R)
Cytochrome b-c1 complex subunit 8polymer819652.12UniProt (Q9CQ69)
Pfam (PF02939)
Mus musculus (house mouse)Complex III subunit 8,Complex III subunit VIII,Ubiquinol-cytochrome c reductase complex 9.5 kDa protein,Ubiquinol-cytochrome c reductase complex ubiquinone-binding protein QP-C
47JC, TC
(3H, 3S)
Cytochrome b-c1 complex subunit 6, mitochondrialpolymer769002.02UniProt (P99028)
Pfam (PF02320)
Mus musculus (house mouse)Complex III subunit 6,Complex III subunit VIII,Cytochrome c1 non-heme 11 kDa protein,Mitochondrial hinge protein,Ubiquinol-cytochrome c reductase complex 11 kDa protein
48KC, VC
(3J, 3U)
Cytochrome b-c1 complex subunit 9polymer637326.32UniProt (Q8R1I1)
Pfam (PF05365)
Mus musculus (house mouse)Complex III subunit 9,Complex III subunit X,Cytochrome c1 non-heme 7 kDa protein,Ubiquinol-cytochrome c reductase complex 7.2 kDa protein
49LC, WC
(3K, 3V)
Cytochrome b-c1 complex subunit 10polymer566546.62UniProt (Q9CPX8)
Pfam (PF08997)
Mus musculus (house mouse)Complex III subunit 10,Complex III subunit XI,Ubiquinol-cytochrome c reductase complex 6.4 kDa protein
50UC
(3T)
Cytochrome b-c1 complex subunit Rieske, mitochondrialpolymer787900.21UniProt (Q9CR68)
Pfam (PF09165)
Mus musculus (house mouse)
51CD, XC
(V1, v1)
NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrialpolymer45750132.32UniProt (Q91YT0)
Pfam (PF01512)
Pfam (PF22461)
Pfam (PF10589)
Mus musculus (house mouse)Complex I-51kD,CI-51kD,NADH-ubiquinone oxidoreductase 51 kDa subunit
52DD, YC
(V2, v2)
NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrialpolymer24426948.92UniProt (Q9D6J6)
Pfam (PF01257)
Mus musculus (house mouse)NADH-ubiquinone oxidoreductase 24 kDa subunit
53ED, ZC
(S1, s1)
NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrialpolymer71678781.52UniProt (Q91VD9)
Pfam (PF13510)
Pfam (PF10588)
Pfam (PF22117)
Pfam (PF22151)
Pfam (PF00384)
Pfam (PF09326)
Mus musculus (house mouse)Complex I-75kD,CI-75kD
54AD, FD
(S7, s7)
NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrialpolymer22424715.92UniProt (Q9DC70)
Pfam (PF01058)
Mus musculus (house mouse)Complex I-20kD,CI-20kD,NADH-ubiquinone oxidoreductase 20 kDa subunit
55BD, GD
(S8, s8)
NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrialpolymer21224068.42UniProt (Q8K3J1)
Pfam (PF12838)
Mus musculus (house mouse)Complex I-23kD,CI-23kD,NADH-ubiquinone oxidoreductase 23 kDa subunit
56HD, OD
(AL, al)
MAGNESIUM IONnon-polymer24.32Chemie (MG)
57ID, PD
(AL, al)
2'-DEOXYGUANOSINE-5'-TRIPHOSPHATEnon-polymer507.22Chemie (DGT)
58JD, QD
(A9, a9)
NADPH DIHYDRO-NICOTINAMIDE-ADENINE-DINUCLEOTIDE PHOSPHATEnon-polymer745.42Chemie (NDP)
59KD, RD
(S6, s6)
ZINC IONnon-polymer65.42Chemie (ZN)
60LD, MD, ND, SD
(A6, B9, 5a, a6)
~{S}-[2-[3-[[(2~{R})-3,3-dimethyl-2-oxidanyl-4-phosphonooxy-butanoyl]amino]propanoylamino]ethyl] (3~{S})-3-oxidanyltetradecanethioatenon-polymer584.74Chemie (EHZ)
61TD, UD, WD, XD
(3C, 3N)
PROTOPORPHYRIN IX CONTAINING FEnon-polymer616.54Chemie (HEM)
62VD, YD
(3D, 3O)
HEME Cnon-polymer618.52Chemie (HEC)
63IE, ZD
(v1, V1)
FLAVIN MONONUCLEOTIDEnon-polymer456.32Chemie (FMN)
64AE, CE, DE, FE, GE...
(V1, S1, S7, S8, v1...)
IRON/SULFUR CLUSTERnon-polymer351.612Chemie (SF4)
65BE, EE, KE, NE
(V2, S1, v2, s1)
FE2/S2 (INORGANIC) CLUSTERnon-polymer175.84Chemie (FES)
Sequence viewer
Contents of the asymmetric unit
PolymersNumber of chains111
Total formula weight2563286.1
Non-Polymers*Number of molecules36
Total formula weight14562.0
All*Total formula weight2577848.2
*Water molecules are not included.

239149

PDB entries from 2025-07-23

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