Formation of I2+III2 supercomplex rescues respiratory chain defects
Resolution: 3.7 Å
EM Method: Single-particle
Fitted PDBs: 8uca
Q-score: 0.411
Liang C, Padavannil A, Zhang S, Beh S, Robinson DRL, Meisterknecht J, Cabrera-Orefice A, Koves TR, Watanabe C, Watanabe M, Illescas M, Lim R, Johnson JM, Ren S, Wu YJ, Kappei D, Ghelli AM, Funai K, Osaka H, Muoio D, Ugalde C, Wittig I, Stroud DA, Letts JA, Ho L
Cell Metab. (2025) 37 pp. 441-459.e11 [ DOI: doi:10.1016/j.cmet.2024.11.011 Pubmed: 39788125 ]
- Nadh dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial (13 kDa, Protein from Mus musculus)
- Fe2/s2 (inorganic) cluster (175 Da, Ligand)
- Nadh dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 (20 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 (10 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial (26 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial (21 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial (42 kDa, Protein from Mus musculus)
- Cytochrome b-c1 complex subunit rieske, mitochondrial (7 kDa, Protein from Mus musculus)
- Heme c (618 Da, Ligand)
- Cytochrome b-c1 complex subunit 8 (9 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial (50 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 (8 kDa, Protein from Mus musculus)
- Nadh-ubiquinone oxidoreductase chain 3 (13 kDa, Protein from Mus musculus)
- Cytochrome c1, heme protein, mitochondrial (27 kDa, Protein from Mus musculus)
- Nadh-ubiquinone oxidoreductase chain 5 (68 kDa, Protein from Mus musculus)
- Nadh-ubiquinone oxidoreductase chain 2 (38 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial (21 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial (24 kDa, Protein from Mus musculus)
- Nadh-ubiquinone oxidoreductase chain 4l (10 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial (11 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 (22 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial (11 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial (24 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 subunit c1, mitochondrial (8 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 (16 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial (40 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 (21 kDa, Protein from Mus musculus)
- Cytochrome b-c1 complex subunit 1, mitochondrial (49 kDa, Protein from Mus musculus)
- Nadh-ubiquinone oxidoreductase chain 6 (18 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 (15 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 (6 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial (52 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 (15 kDa, Protein from Mus musculus)
- Cytochrome b-c1 complex subunit 9 (21 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 subunit c2 (14 kDa, Protein from Mus musculus)
- Mitochondrial super-complex with ci dimer and ciii dimer (super-complex xl) (Complex from Mus musculus)
- Nadh-ubiquinone oxidoreductase 75 kda subunit, mitochondrial (78 kDa, Protein from Mus musculus)
- Magnesium ion (24 Da, Ligand)
- Cytochrome b-c1 complex subunit 6, mitochondrial (9 kDa, Protein from Mus musculus)
- Nadph dihydro-nicotinamide-adenine-dinucleotide phosphate (745 Da, Ligand)
- Acyl carrier protein, mitochondrial (17 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 (9 kDa, Protein from Mus musculus)
- Cytochrome b-c1 complex subunit 10 (6 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 (13 kDa, Protein from Mus musculus)
- Cytochrome b-c1 complex subunit 7 (13 kDa, Protein from Mus musculus)
- 2'-deoxyguanosine-5'-triphosphate (507 Da, Ligand)
- Zinc ion (65 Da, Ligand)
- Nadh dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial (30 kDa, Protein from Mus musculus)
- Nadh-ubiquinone oxidoreductase chain 4 (51 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 (11 kDa, Protein from Mus musculus)
- Cytochrome b-c1 complex subunit 9 (7 kDa, Protein from Mus musculus)
- Cytochrome b (43 kDa, Protein from Mus musculus)
- Flavin mononucleotide (456 Da, Ligand)
- Protoporphyrin ix containing fe (616 Da, Ligand)
- Nadh dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 (14 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 (12 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 (17 kDa, Protein from Mus musculus)
- Nadh-ubiquinone oxidoreductase chain 1 (36 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial (17 kDa, Protein from Mus musculus)
- ~{s}-[2-[3-[[(2~{r})-3,3-dimethyl-2-oxidanyl-4-phosphonooxy-butanoyl]amino]propanoylamino]ethyl] (3~{s})-3-oxidanyltetradecanethioate (584 Da, Ligand)
- Nadh dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 (15 kDa, Protein from Mus musculus)
- Iron/sulfur cluster (351 Da, Ligand)
- Cytochrome b-c1 complex subunit 2, mitochondrial (46 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial (19 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 (16 kDa, Protein from Mus musculus)
- Nadh dehydrogenase [ubiquinone] iron-sulfur protein 5 (12 kDa, Protein from Mus musculus)