8UCA

Formation of I2+III2 supercomplex rescues respiratory chain defects


Experimental Data Snapshot

  • Method: ELECTRON MICROSCOPY
  • Resolution: 3.70 Å
  • Aggregation State: PARTICLE 
  • Reconstruction Method: SINGLE PARTICLE 

wwPDB Validation   3D Report Full Report


This is version 1.0 of the entry. See complete history


Literature

Formation of I2+III2 supercomplex rescues respiratory chain defects

Letts, J.A.Padavannil, A.

(2024) Cell Metab 


Macromolecules
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Entity ID: 1
MoleculeChains Sequence LengthOrganismDetailsImage
NADH-ubiquinone oxidoreductase chain 3A [auth 3],
OA [auth 3a]
115Mus musculusMutation(s): 0 
Gene Names: mt-Nd3
EC: 7.1.1.2
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Entity ID: 2
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrialB [auth S3],
PA [auth s3]
263Mus musculusMutation(s): 0 
Gene Names: Ndufs3
EC: 7.1.1.2
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IMPC:  MGI:1915599
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Entity ID: 3
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrialC [auth S2],
QA [auth s2]
463Mus musculusMutation(s): 0 
Gene Names: Ndufs2
EC: 7.1.1.2
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IMPC:  MGI:2385112
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Entity ID: 4
MoleculeChains Sequence LengthOrganismDetailsImage
NADH-ubiquinone oxidoreductase chain 1D [auth 1],
RA [auth 1a]
318Mus musculusMutation(s): 0 
Gene Names: mt-Nd1ND1
EC: 7.1.1.2
UniProt
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Entity ID: 5
MoleculeChains Sequence LengthOrganismDetailsImage
NADH-ubiquinone oxidoreductase chain 6E [auth 6],
SA [auth 6a]
172Mus musculusMutation(s): 0 
Gene Names: Mtnd6mt-Nd6Nd6
EC: 7.1.1.2
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Entity ID: 6
MoleculeChains Sequence LengthOrganismDetailsImage
NADH-ubiquinone oxidoreductase chain 4LF [auth 4L],
TA [auth 4l]
98Mus musculusMutation(s): 0 
Gene Names: Mtnd4lmt-Nd4lNd4l
EC: 7.1.1.2
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Entity ID: 7
MoleculeChains Sequence LengthOrganismDetailsImage
NADH-ubiquinone oxidoreductase chain 5G [auth 5],
UA [auth 5a]
607Mus musculusMutation(s): 0 
Gene Names: Mtnd5mt-Nd5Nd5
EC: 7.1.1.2
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Entity ID: 8
MoleculeChains Sequence LengthOrganismDetailsImage
NADH-ubiquinone oxidoreductase chain 4H [auth 4],
VA [auth 4a]
459Mus musculusMutation(s): 0 
Gene Names: Mtnd4mt-Nd4Nd4
EC: 7.1.1.2
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Entity ID: 9
MoleculeChains Sequence LengthOrganismDetailsImage
NADH-ubiquinone oxidoreductase chain 2I [auth 2],
WA [auth 2a]
345Mus musculusMutation(s): 0 
Gene Names: mt-Nd2Nd2
EC: 7.1.1.2
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Entity ID: 10
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrialJ [auth AL],
XA [auth al]
355Mus musculusMutation(s): 0 
Gene Names: Ndufa10
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IMPC:  MGI:1914523
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Entity ID: 11
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrialK [auth A9],
YA [auth a9]
377Mus musculusMutation(s): 0 
Gene Names: Ndufa9
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IMPC:  MGI:1913358
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Entity ID: 12
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrialL [auth S4],
ZA [auth s4]
175Mus musculusMutation(s): 0 
Gene Names: Ndufs4
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Entity ID: 13
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrialAB [auth s6],
M [auth S6]
116Mus musculusMutation(s): 0 
Gene Names: Ndufs6Ip13
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IMPC:  MGI:107932
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Entity ID: 14
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2BB [auth a2],
N [auth A2]
99Mus musculusMutation(s): 0 
Gene Names: Ndufa2
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Find proteins for Q9CQ75 (Mus musculus)
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Entity ID: 15
MoleculeChains Sequence LengthOrganismDetailsImage
Acyl carrier protein, mitochondrialCB [auth ab],
DB [auth ac],
O [auth AB],
P [auth AC]
156Mus musculusMutation(s): 0 
Gene Names: Ndufab1
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Entity ID: 16
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5EB [auth a5],
Q [auth A5]
116Mus musculusMutation(s): 0 
Gene Names: Ndufa5
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Entity ID: 17
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6FB [auth a6],
R [auth A6]
131Mus musculusMutation(s): 0 
Gene Names: Ndufa6
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IMPC:  MGI:1914380
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Entity ID: 18
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8GB [auth a8],
S [auth A8]
172Mus musculusMutation(s): 0 
Gene Names: Ndufa8
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IMPC:  MGI:1915625
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Entity ID: 19
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11HB [auth am],
T [auth AM]
142Mus musculusMutation(s): 0 
Gene Names: Ndufa11
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IMPC:  MGI:1917125
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Entity ID: 20
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13IB [auth ao],
U [auth AO]
144Mus musculusMutation(s): 0 
Gene Names: Ndufa13Grim19
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Entity ID: 21
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1JB [auth a1],
V [auth A1]
70Mus musculusMutation(s): 0 
Gene Names: Ndufa1
UniProt
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Entity ID: 22
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3KB [auth a3],
W [auth A3]
84Mus musculusMutation(s): 0 
Gene Names: Ndufa3
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Entity ID: 23
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrialLB [auth c1],
X [auth C1]
76Mus musculusMutation(s): 0 
Gene Names: Ndufc1
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Entity ID: 24
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 subunit C2MB [auth c2],
Y [auth C2]
120Mus musculusMutation(s): 0 
Gene Names: Ndufc2
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Entity ID: 25
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] iron-sulfur protein 5NB [auth s5],
Z [auth S5]
106Mus musculusMutation(s): 0 
Gene Names: Ndufs5
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IMPC:  MGI:1890889
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Entity ID: 26
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1AA [auth B1],
OB [auth b1]
57Mus musculusMutation(s): 0 
Gene Names: Ndufb1
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Entity ID: 27
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrialBA [auth BM],
PB [auth bm]
151Mus musculusMutation(s): 0 
Gene Names: Ndufb11Np15
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Entity ID: 28
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrialCA [auth B5],
QB [auth b5]
189Mus musculusMutation(s): 0 
Gene Names: Ndufb5
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IMPC:  MGI:1913296
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Entity ID: 29
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6DA [auth B6],
RB [auth b6]
127Mus musculusMutation(s): 0 
Gene Names: Ndufb6Gm137
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Entity ID: 30
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrialEA [auth B2],
SB [auth b2]
105Mus musculusMutation(s): 0 
Gene Names: Ndufb2
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Find proteins for Q9CPU2 (Mus musculus)
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Entity ID: 31
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3FA [auth B3],
TB [auth b3]
104Mus musculusMutation(s): 0 
Gene Names: Ndufb3
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Entity ID: 32
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrialGA [auth B8],
UB [auth b8]
186Mus musculusMutation(s): 0 
Gene Names: Ndufb8
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IMPC:  MGI:1914514
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Entity ID: 33
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4HA [auth B4],
VB [auth b4]
129Mus musculusMutation(s): 0 
Gene Names: Ndufb4
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Entity ID: 34
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9IA [auth B9],
WB [auth b9]
179Mus musculusMutation(s): 0 
Gene Names: Ndufb9
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IMPC:  MGI:1913468
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Entity ID: 35
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7JA [auth B7],
XB [auth b7]
136Mus musculusMutation(s): 0 
Gene Names: Ndufb7
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Entity ID: 36
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10KA [auth BL],
YB [auth bl]
176Mus musculusMutation(s): 0 
Gene Names: Ndufb10
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Find proteins for Q9DCS9 (Mus musculus)
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Entity ID: 37
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12LA [auth AN],
ZB [auth an]
145Mus musculusMutation(s): 0 
Gene Names: Ndufa12
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Find proteins for Q7TMF3 (Mus musculus)
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Entity ID: 38
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7AC [auth a7],
MA [auth A7]
112Mus musculusMutation(s): 0 
Gene Names: Ndufa7
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IMPC:  MGI:1913666
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Entity ID: 39
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrialBC [auth v3],
NA [auth V3]
104Mus musculusMutation(s): 0 
Gene Names: Ndufv3
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IMPC:  MGI:1890894
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Entity ID: 40
MoleculeChains Sequence LengthOrganismDetailsImage
Cytochrome b-c1 complex subunit 1, mitochondrialCC [auth 3A],
MC [auth 3L]
446Mus musculusMutation(s): 0 
Gene Names: Uqcrc1
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IMPC:  MGI:107876
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Entity ID: 41
MoleculeChains Sequence LengthOrganismDetailsImage
Cytochrome b-c1 complex subunit 2, mitochondrialDC [auth 3B],
NC [auth 3M]
439Mus musculusMutation(s): 0 
Gene Names: Uqcrc2
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Entity ID: 42
MoleculeChains Sequence LengthOrganismDetailsImage
Cytochrome bEC [auth 3C],
OC [auth 3N]
380Mus musculusMutation(s): 0 
Gene Names: Mt-Cyb
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Entity ID: 43
MoleculeChains Sequence LengthOrganismDetailsImage
Cytochrome c1, heme protein, mitochondrialFC [auth 3D],
PC [auth 3O]
241Mus musculusMutation(s): 0 
Gene Names: Cyc1
EC: 7.1.1.8
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IMPC:  MGI:1913695
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Entity ID: 44
MoleculeChains Sequence LengthOrganismDetailsImage
Cytochrome b-c1 complex subunit 9GC [auth 3E],
QC [auth 3P]
196Mus musculusMutation(s): 0 
Gene Names: Uqcrfs1
EC: 7.1.1.8
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Entity ID: 45
MoleculeChains Sequence LengthOrganismDetailsImage
Cytochrome b-c1 complex subunit 7HC [auth 3F],
RC [auth 3Q]
110Mus musculusMutation(s): 0 
Gene Names: Uqcrb
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IMPC:  MGI:1914780
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Entity ID: 46
MoleculeChains Sequence LengthOrganismDetailsImage
Cytochrome b-c1 complex subunit 8IC [auth 3G],
SC [auth 3R]
81Mus musculusMutation(s): 0 
Gene Names: Uqcrq
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IMPC:  MGI:107807
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Entity ID: 47
MoleculeChains Sequence LengthOrganismDetailsImage
Cytochrome b-c1 complex subunit 6, mitochondrialJC [auth 3H],
TC [auth 3S]
76Mus musculusMutation(s): 0 
Gene Names: Uqcrh
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IMPC:  MGI:1913826
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Entity ID: 48
MoleculeChains Sequence LengthOrganismDetailsImage
Cytochrome b-c1 complex subunit 9KC [auth 3J],
VC [auth 3U]
63Mus musculusMutation(s): 0 
Gene Names: Uqcr10
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IMPC:  MGI:1913402
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Entity ID: 49
MoleculeChains Sequence LengthOrganismDetailsImage
Cytochrome b-c1 complex subunit 10LC [auth 3K],
WC [auth 3V]
56Mus musculusMutation(s): 0 
Gene Names: Uqcr11Uqcr
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Entity ID: 50
MoleculeChains Sequence LengthOrganismDetailsImage
Cytochrome b-c1 complex subunit Rieske, mitochondrialUC [auth 3T]78Mus musculusMutation(s): 0 
Gene Names: Uqcrfs1
EC: 7.1.1.8
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Entity ID: 51
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrialCD [auth v1],
XC [auth V1]
457Mus musculusMutation(s): 0 
Gene Names: Ndufv1
EC: 7.1.1.2
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Find proteins for Q91YT0 (Mus musculus)
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IMPC:  MGI:107851
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Entity ID: 52
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrialDD [auth v2],
YC [auth V2]
244Mus musculusMutation(s): 0 
Gene Names: Ndufv2
EC: 7.1.1.2
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IMPC:  MGI:1920150
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Entity ID: 53
MoleculeChains Sequence LengthOrganismDetailsImage
NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrialED [auth s1],
ZC [auth S1]
716Mus musculusMutation(s): 0 
Gene Names: Ndufs1
EC: 7.1.1.2
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IMPC:  MGI:2443241
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Entity ID: 54
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrialAD [auth S7],
FD [auth s7]
224Mus musculusMutation(s): 0 
Gene Names: Ndufs7
EC: 7.1.1.2
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IMPC:  MGI:1922656
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Entity ID: 55
MoleculeChains Sequence LengthOrganismDetailsImage
NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrialBD [auth S8],
GD [auth s8]
212Mus musculusMutation(s): 0 
Gene Names: Ndufs8
EC: 7.1.1.2
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IMPC:  MGI:2385079
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Small Molecules
Ligands 10 Unique
IDChains Name / Formula / InChI Key2D Diagram3D Interactions
NDP
Query on NDP

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JD [auth A9],
QD [auth a9]
NADPH DIHYDRO-NICOTINAMIDE-ADENINE-DINUCLEOTIDE PHOSPHATE
C21 H30 N7 O17 P3
ACFIXJIJDZMPPO-NNYOXOHSSA-N
HEC
Query on HEC

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VD [auth 3D],
YD [auth 3O]
HEME C
C34 H34 Fe N4 O4
HXQIYSLZKNYNMH-LJNAALQVSA-N
HEM
Query on HEM

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TD [auth 3C],
UD [auth 3C],
WD [auth 3N],
XD [auth 3N]
PROTOPORPHYRIN IX CONTAINING FE
C34 H32 Fe N4 O4
KABFMIBPWCXCRK-RGGAHWMASA-L
EHZ
Query on EHZ

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LD [auth A6],
MD [auth B9],
ND [auth 5a],
SD [auth a6]
~{S}-[2-[3-[[(2~{R})-3,3-dimethyl-2-oxidanyl-4-phosphonooxy-butanoyl]amino]propanoylamino]ethyl] (3~{S})-3-oxidanyltetradecanethioate
C25 H49 N2 O9 P S
JYSKQPQRUCZFIQ-REWPJTCUSA-N
DGT
Query on DGT

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ID [auth AL],
PD [auth al]
2'-DEOXYGUANOSINE-5'-TRIPHOSPHATE
C10 H16 N5 O13 P3
HAAZLUGHYHWQIW-KVQBGUIXSA-N
FMN
Query on FMN

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IE [auth v1],
ZD [auth V1]
FLAVIN MONONUCLEOTIDE
C17 H21 N4 O9 P
FVTCRASFADXXNN-SCRDCRAPSA-N
SF4
Query on SF4

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AE [auth V1]
CE [auth S1]
DE [auth S1]
FE [auth S7]
GE [auth S8]
AE [auth V1],
CE [auth S1],
DE [auth S1],
FE [auth S7],
GE [auth S8],
HE [auth S8],
JE [auth v1],
LE [auth s1],
ME [auth s1],
OE [auth s7],
PE [auth s8],
QE [auth s8]
IRON/SULFUR CLUSTER
Fe4 S4
LJBDFODJNLIPKO-UHFFFAOYSA-N
FES
Query on FES

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BE [auth V2],
EE [auth S1],
KE [auth v2],
NE [auth s1]
FE2/S2 (INORGANIC) CLUSTER
Fe2 S2
NIXDOXVAJZFRNF-UHFFFAOYSA-N
ZN
Query on ZN

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KD [auth S6],
RD [auth s6]
ZINC ION
Zn
PTFCDOFLOPIGGS-UHFFFAOYSA-N
MG
Query on MG

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HD [auth AL],
OD [auth al]
MAGNESIUM ION
Mg
JLVVSXFLKOJNIY-UHFFFAOYSA-N
Modified Residues  2 Unique
IDChains TypeFormula2D DiagramParent
2MR
Query on 2MR
C [auth S2],
QA [auth s2]
L-PEPTIDE LINKINGC8 H18 N4 O2ARG
FME
Query on FME
D [auth 1],
RA [auth 1a]
L-PEPTIDE LINKINGC6 H11 N O3 SMET
Experimental Data & Validation

Experimental Data

  • Method: ELECTRON MICROSCOPY
  • Resolution: 3.70 Å
  • Aggregation State: PARTICLE 
  • Reconstruction Method: SINGLE PARTICLE 
EM Software:
TaskSoftware PackageVersion
MODEL REFINEMENTPHENIX1.20.1_4487:

Structure Validation

View Full Validation Report



Entry History & Funding Information

Deposition Data


Funding OrganizationLocationGrant Number
National Institutes of Health/National Institute of General Medical Sciences (NIH/NIGMS)United StatesR35GM137929

Revision History  (Full details and data files)

  • Version 1.0: 2025-01-15
    Type: Initial release