Structure analysis

Cryo-EM structure of human respiratory supercomplex I1III2IV1

Electron Microscopy
3.9Å resolution
Source organisms:
Assembly composition:
hetero 80-mer (preferred)
Entry contents: 68 distinct polypeptide molecules

Assemblies

Assembly 1 (preferred)
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Multimeric state: hetero 80-mer
PDBe Complex ID: PDB-CPX-110813

Macromolecules

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Chain: B
Length: 176 amino acids
Theoretical weight: 20.31 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O00217 (Residues: 35-210; Coverage: 84%)
Gene name: NDUFS8
Pfam: 4Fe-4S dicluster domain
InterPro:

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Chain: C
Length: 156 amino acids
Theoretical weight: 17.89 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O75251 (Residues: 58-213; Coverage: 73%)
Gene name: NDUFS7
Pfam: NADH ubiquinone oxidoreductase, 20 Kd subunit
InterPro:

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Chain: E
Length: 113 amino acids
Theoretical weight: 13.76 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: P56556 (Residues: 16-128; Coverage: 88%)
Gene names: LYRM6, NADHB14, NDUFA6
Pfam: Complex1_LYR-like
InterPro:

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Chains: G, X
Length: 85 amino acids
Theoretical weight: 9.85 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O14561 (Residues: 72-156; Coverage: 55%)
Gene name: NDUFAB1
Pfam: Phosphopantetheine attachment site
InterPro:

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Chain: H
Length: 112 amino acids
Theoretical weight: 13.12 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: Q16718 (Residues: 5-116; Coverage: 97%)
Gene name: NDUFA5
Pfam: ETC complex I subunit conserved region
InterPro: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5

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Chain: I
Length: 110 amino acids
Theoretical weight: 12.28 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O95182 (Residues: 4-113; Coverage: 97%)
Gene name: NDUFA7
Pfam: NADH:ubiquinone oxidoreductase subunit B14.5a (Complex I-B14.5a)
InterPro: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7

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Chain: J
Length: 337 amino acids
Theoretical weight: 38.39 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: Q16795 (Residues: 40-376; Coverage: 89%)
Gene names: NDUFA9, NDUFS2L
Pfam: NAD dependent epimerase/dehydratase family
InterPro:

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Chain: K
Length: 33 amino acids
Theoretical weight: 3.9 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: P56181 (Residues: 74-106; Coverage: 31%)
Gene name: NDUFV3
Pfam: NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial
InterPro: NADH-ubiquinone oxidoreductase flavoprotein 3

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Chain: L
Length: 118 amino acids
Theoretical weight: 13.72 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O43181 (Residues: 58-175; Coverage: 67%)
Gene name: NDUFS4
Pfam: NADH dehydrogenase ubiquinone Fe-S protein 4
InterPro:

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Chain: N
Length: 143 amino acids
Theoretical weight: 16.88 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: Q9UI09 (Residues: 2-144; Coverage: 99%)
Gene names: DAP13, NDUFA12
Pfam: NADH ubiquinone oxidoreductase subunit NDUFA12
InterPro: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12

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Chain: O
Length: 212 amino acids
Theoretical weight: 23.43 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: P19404 (Residues: 36-247; Coverage: 85%)
Gene name: NDUFV2
Pfam: Thioredoxin-like [2Fe-2S] ferredoxin
InterPro:

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Chain: S
Length: 70 amino acids
Theoretical weight: 8.08 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O15239 (Residues: 1-70; Coverage: 100%)
Gene name: NDUFA1
Pfam: NADH-ubiquinone oxidoreductase MWFE subunit
InterPro: NADH dehydrogenase [ubiquinone] (complex I), alpha subcomplex subunit 1

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Chain: T
Length: 95 amino acids
Theoretical weight: 10.58 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O75380 (Residues: 29-123; Coverage: 77%)
Gene name: NDUFS6
Pfam: Zinc-finger domain
InterPro:

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Chain: U
Length: 83 amino acids
Theoretical weight: 9.16 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O95167 (Residues: 2-84; Coverage: 99%)
Gene name: NDUFA3
Pfam: NADH dehydrogenase 1 alpha subcomplex subunit 3
InterPro: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3

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Chain: V
Length: 140 amino acids
Theoretical weight: 14.74 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: Q86Y39 (Residues: 2-141; Coverage: 99%)
Gene name: NDUFA11
InterPro: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11

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Chain: W
Length: 138 amino acids
Theoretical weight: 16.13 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: Q9P0J0 (Residues: 7-144; Coverage: 96%)
Gene names: CDA016, CGI-39, GRIM19, NDUFA13
Pfam: GRIM-19 protein
InterPro: GRIM-19

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Chain: Y
Length: 59 amino acids
Theoretical weight: 7.47 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O95178 (Residues: 39-97; Coverage: 56%)
Gene name: NDUFB2
Pfam: NADH dehydrogenase 1 beta subcomplex subunit 2
InterPro: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, animal type

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Chain: Z
Length: 80 amino acids
Theoretical weight: 9.26 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O43676 (Residues: 10-89; Coverage: 82%)
Gene name: NDUFB3
Pfam: NADH-ubiquinone oxidoreductase B12 subunit family
InterPro: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3

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Chain: a
Length: 138 amino acids
Theoretical weight: 16.57 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O43674 (Residues: 52-189; Coverage: 73%)
Gene name: NDUFB5
Pfam: NADH:ubiquinone oxidoreductase, NDUFB5/SGDH subunit
InterPro: NADH:ubiquinone oxidoreductase, NDUFB5/SGDH subunit

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Chain: b
Length: 124 amino acids
Theoretical weight: 15.01 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O95139 (Residues: 3-126; Coverage: 97%)
Gene name: NDUFB6
Pfam: NADH:ubiquinone oxidoreductase, NDUFB6/B17 subunit
InterPro: NADH dehydrogenase 1, beta subcomplex, subunit 6

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Chain: c
Length: 153 amino acids
Theoretical weight: 18.27 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O95169 (Residues: 34-186; Coverage: 82%)
Gene name: NDUFB8
Pfam: NADH-ubiquinone oxidoreductase ASHI subunit (CI-ASHI or NDUFB8)
InterPro:

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Chain: d
Length: 171 amino acids
Theoretical weight: 20.72 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O96000 (Residues: 1-171; Coverage: 99%)
Gene name: NDUFB10
Pfam: NADH-ubiquinone oxidoreductase subunit 10
InterPro:

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Chain: e
Length: 97 amino acids
Theoretical weight: 11.49 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: Q9NX14 (Residues: 54-150; Coverage: 63%)
Gene names: NDUFB11, UNQ111/PRO1064
Pfam: ESSS subunit of NADH:ubiquinone oxidoreductase (complex I)
InterPro: NADH:ubiquinone oxidoreductase, ESSS subunit

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Chain: f
Length: 47 amino acids
Theoretical weight: 5.7 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O43677 (Residues: 28-74; Coverage: 62%)
Gene name: NDUFC1
Pfam: NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial
InterPro: NADH-ubiquinone oxidoreductase 1 subunit C1

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Chain: g
Length: 119 amino acids
Theoretical weight: 14.21 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O95298 (Residues: 1-119; Coverage: 100%)
Gene names: HLC1, NDUFC2
Pfam: NADH-ubiquinone oxidoreductase subunit b14.5b (NDUFC2)
InterPro: NADH dehydrogenase [ubiquinone] 1 subunit C2, NDUC2

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Chain: h
Length: 104 amino acids
Theoretical weight: 12.31 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O43920 (Residues: 2-105; Coverage: 98%)
Gene name: NDUFS5
Pfam: NADH:ubiquinone oxidoreductase, NDUFS5-15kDa
InterPro: NADH:ubiquinone oxidoreductase, iron-sulphur subunit 5

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Chain: i
Length: 347 amino acids
Theoretical weight: 39.01 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: P03891 (Residues: 1-347; Coverage: 100%)
Gene names: MT-ND2, MTND2, NADH2, ND2
Pfam:
InterPro:

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Chain: j
Length: 115 amino acids
Theoretical weight: 13.15 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: B9EE38 (Residues: 1-115; Coverage: 99%)
Gene name: NADH3
Pfam: NADH-ubiquinone/plastoquinone oxidoreductase, chain 3
InterPro:

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Chain: k
Length: 97 amino acids
Theoretical weight: 10.7 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: V9JN72 (Residues: 1-97; Coverage: 99%)
Gene name: ND4L
Pfam: NADH-ubiquinone/plastoquinone oxidoreductase chain 4L
InterPro:

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Chain: m
Length: 174 amino acids
Theoretical weight: 18.66 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: Q8HAX7 (Residues: 1-174; Coverage: 100%)
Gene name: ND6
Pfam: NADH-ubiquinone/plastoquinone oxidoreductase chain 6
InterPro: NADH:ubiquinone/plastoquinone oxidoreductase, chain 6

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Chain: n
Length: 56 amino acids
Theoretical weight: 6.74 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O75438 (Residues: 3-58; Coverage: 97%)
Gene name: NDUFB1
Pfam: MNLL subunit
InterPro: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1, NDUB1

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Chain: o
Length: 128 amino acids
Theoretical weight: 15.1 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O95168 (Residues: 2-129; Coverage: 99%)
Gene name: NDUFB4
Pfam: NADH-ubiquinone oxidoreductase B15 subunit (NDUFB4)
InterPro: NADH:ubiquinone oxidoreductase, subunit NDUFB4

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Chain: p
Length: 172 amino acids
Theoretical weight: 21.19 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: Q9Y6M9 (Residues: 8-179; Coverage: 96%)
Gene names: LYRM3, NDUFB9, UQOR22
Pfam: Complex 1 protein (LYR family)
InterPro:

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Chain: s
Length: 318 amino acids
Theoretical weight: 35.62 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: H9PGF0 (Residues: 1-318; Coverage: 100%)
Gene name: ND1
Pfam: NADH dehydrogenase
InterPro:

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Chain: u
Length: 169 amino acids
Theoretical weight: 19.85 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: P51970 (Residues: 4-172; Coverage: 98%)
Gene name: NDUFA8
Pfam: CHCH domain
InterPro:

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Chain: v
Length: 122 amino acids
Theoretical weight: 15 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: P17568 (Residues: 3-124; Coverage: 89%)
Gene name: NDUFB7
Pfam: NADH-ubiquinone oxidoreductase B18 subunit (NDUFB7)
InterPro: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7, NDUB7

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Chain: w
Length: 320 amino acids
Theoretical weight: 37.2 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O95299 (Residues: 36-355; Coverage: 90%)
Gene name: NDUFA10
Pfam: Deoxynucleoside kinase
InterPro:

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Chain: 0
Length: 144 amino acids
Theoretical weight: 16.91 KDa
Source organism: Bos taurus
UniProt:
  • Canonical: P00423 (Residues: 26-169; Coverage: 85%)
Gene names: COX4, COX4I1
Pfam: Cytochrome c oxidase subunit IV
InterPro:

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Chain: 1
Length: 109 amino acids
Theoretical weight: 12.45 KDa
Source organism: Bos taurus
UniProt:
  • Canonical: P00426 (Residues: 44-152; Coverage: 72%)
Gene name: COX5A
Pfam: Cytochrome c oxidase subunit Va
InterPro:

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Chain: 2
Length: 98 amino acids
Theoretical weight: 10.68 KDa
Source organism: Bos taurus
UniProt:
  • Canonical: P00428 (Residues: 32-129; Coverage: 76%)
Gene name: COX5B
Pfam: Cytochrome c oxidase subunit Vb
InterPro:

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Chain: 3
Length: 84 amino acids
Theoretical weight: 9.45 KDa
Source organism: Bos taurus
UniProt:
  • Canonical: P07471 (Residues: 13-96; Coverage: 87%)
Gene names: COX6A, COX6A2
Pfam: Cytochrome c oxidase subunit VIa
InterPro:

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Chain: 4
Length: 75 amino acids
Theoretical weight: 8.93 KDa
Source organism: Bos taurus
UniProt:
  • Canonical: P00429 (Residues: 12-86; Coverage: 87%)
Gene names: COX6B, COX6B1
Pfam: Cytochrome oxidase c subunit VIb
InterPro:

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Chain: 5
Length: 73 amino acids
Theoretical weight: 8.49 KDa
Source organism: Bos taurus
UniProt:
  • Canonical: P04038 (Residues: 2-74; Coverage: 99%)
Gene name: COX6C
Pfam: Cytochrome c oxidase subunit VIc
InterPro:

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Chain: 6
Length: 56 amino acids
Theoretical weight: 6.29 KDa
Source organism: Bos taurus
UniProt:
  • Canonical: P07470 (Residues: 22-77; Coverage: 70%)
Gene names: COX7A, COX7A1, COX7AH
Pfam: Cytochrome c oxidase subunit VII
InterPro:

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Chain: 7
Length: 49 amino acids
Theoretical weight: 5.44 KDa
Source organism: Bos taurus
UniProt:
  • Canonical: P13183 (Residues: 30-78; Coverage: 61%)
Gene name: COX7B
Pfam: Cytochrome C oxidase chain VIIB
InterPro:

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Chain: 8
Length: 47 amino acids
Theoretical weight: 5.45 KDa
Source organism: Bos taurus
UniProt:
  • Canonical: P00430 (Residues: 17-63; Coverage: 75%)
Gene names: COX7C, COX7CP1
Pfam: Cytochrome c oxidase subunit VIIc
InterPro:

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Chain: 9
Length: 43 amino acids
Theoretical weight: 4.74 KDa
Source organism: Bos taurus
UniProt:
  • Canonical: P10175 (Residues: 25-67; Coverage: 61%)
Gene names: COX8B, COX8H
Pfam: Cytochrome oxidase c subunit VIII
InterPro:

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Chains: AA, AN
Length: 81 amino acids
Theoretical weight: 9.79 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O14949 (Residues: 2-82; Coverage: 99%)
Gene name: UQCRQ
Pfam: UcrQ family
InterPro:

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Chains: AB, AO
Length: 57 amino acids
Theoretical weight: 5.89 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: P47985 (Residues: 1-57; Coverage: 21%)
Gene name: UQCRFS1
Pfam: Ubiquinol-cytochrome c reductase 8 kDa, N-terminal
InterPro:

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Chains: AD, AQ
Length: 62 amino acids
Theoretical weight: 7.19 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: Q9UDW1 (Residues: 2-63; Coverage: 98%)
Gene names: HSPC119, UCRC, UQCR10
Pfam: Ubiquinol-cytochrome C reductase, UQCRX/QCR9 like
InterPro:

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Chains: AE, AR
Length: 74 amino acids
Theoretical weight: 8.86 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: P07919 (Residues: 18-91; Coverage: 81%)
Gene name: UQCRH
Pfam: Ubiquinol-cytochrome C reductase hinge protein
InterPro:

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Chains: AF, AS
Length: 106 amino acids
Theoretical weight: 13.04 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: P14927 (Residues: 6-111; Coverage: 96%)
Gene names: UQBP, UQCRB
Pfam: Ubiquinol-cytochrome C reductase complex 14kD subunit
InterPro:

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Chains: AG, AT
Length: 51 amino acids
Theoretical weight: 5.96 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: O14957 (Residues: 2-52; Coverage: 91%)
Gene names: UQCR, UQCR11
Pfam: Ubiquinol-cytochrome C reductase complex, 6.4kD protein
InterPro:

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Chains: AH, AU
Length: 241 amino acids
Theoretical weight: 27.39 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: P08574 (Residues: 85-325; Coverage: 74%)
Gene name: CYC1
Pfam: Cytochrome C1 family
InterPro:

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Chains: AK, AW
Length: 419 amino acids
Theoretical weight: 44.95 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: P22695 (Residues: 35-453; Coverage: 93%)
Gene name: UQCRC2
Pfam:
InterPro:

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Chains: AL, AY
Length: 446 amino acids
Theoretical weight: 49.18 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: P31930 (Residues: 35-480; Coverage: 93%)
Gene name: UQCRC1
Pfam:
InterPro:

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