Electron transport chain: OXPHOS system in mitochondria

No Pathway Network information available for Electron transport chain: OXPHOS system in mitochondria

Pathways in the Electron transport chain: OXPHOS system in mitochondria SuperPath

#	Name	Source	Genes
1	Electron transport chain: OXPHOS system in mitochondria	WikiPathways	ATP5F1A ATP5F1B ATP5F1C ATP5F1D ATP5F1E ATP5IF1 ATP5MC1 ATP5MC2 ATP5MC3 ATP5ME ATP5MF ATP5MG ATP5PB ATP5PD ATP5PF ATP5PO COX11 COX15 COX17 COX4I1 COX5A COX5B COX6A1 COX6A2 COX6B1 COX6C COX7A1 COX7A2 COX7A2L COX7B COX7C COX8A COXFA4 DMAC2L MT-ATP6 MT-ATP8 MT-CO1 MT-CO2 MT-CO3 MT-CYB MT-ND1 MT-ND2 MT-ND3 MT-ND4 MT-ND4L MT-ND5 MT-ND6 NDUFA1 NDUFA10 NDUFA11 NDUFA12 NDUFA2 NDUFA3 NDUFA5 NDUFA6 NDUFA7 NDUFA8 NDUFA9 NDUFAB1 NDUFB1 NDUFB10 NDUFB2 NDUFB3 NDUFB4 NDUFB5 NDUFB6 NDUFB7 NDUFB8 NDUFB9 NDUFC1 NDUFC2 NDUFS1 NDUFS2 NDUFS3 NDUFS4 NDUFS5 NDUFS6 NDUFS7 NDUFS8 NDUFV1 NDUFV2 NDUFV3 SCO1 SDHA SDHB SDHC SDHD SLC25A14 SLC25A27 SLC25A4 SLC25A5 SLC25A6 SURF1 UCP1 UCP2 UCP3 UQCR10 UQCR11 UQCRB UQCRC1 UQCRC2 UQCRFS1 UQCRH UQCRQ (see all 104) (see less)
2	Oxidative phosphorylation	WikiPathways	ATP5F1A ATP5F1B ATP5F1D ATP5F1E ATP5MC1 ATP5MC2 ATP5MC3 ATP5ME ATP5MF ATP5MG ATP5PB ATP5PD ATP5PF ATP5PO ATP6AP1 ATP6AP2 COXFA4 COXFA4L2 DMAC2L GZMB MT-ATP6 MT-ND1 MT-ND2 MT-ND3 MT-ND4 MT-ND4L MT-ND5 MT-ND6 MTCO3P12 NDUFA10 NDUFA11 NDUFA2 NDUFA3 NDUFA5 NDUFA6 NDUFA7 NDUFA8 NDUFA9 NDUFAB1 NDUFB1 NDUFB10 NDUFB2 NDUFB4 NDUFB5 NDUFB6 NDUFB7 NDUFB8 NDUFB9 NDUFC1 NDUFC2 NDUFS1 NDUFS2 NDUFS3 NDUFS4 NDUFS5 NDUFS6 NDUFS7 NDUFS8 NDUFV1 NDUFV2 NDUFV3 (see all 61) (see less)

Ordered by rank within the SuperPath, via the multiplicity of each gene in the constituent pathways

#	Symbol	Description	Category	# Related Pathways
1	NDUFAB1	NADH:Ubiquinone Oxidoreductase Subunit AB1	Protein Coding	2
2	ATP5PF	ATP Synthase Peripheral Stalk Subunit F6	Protein Coding	2
3	ATP5ME	ATP Synthase Membrane Subunit E	Protein Coding	2
4	ATP5PB	ATP Synthase Peripheral Stalk-Membrane Subunit B	Protein Coding	2
5	ATP5F1E	ATP Synthase F1 Subunit Epsilon	Protein Coding	2
6	ATP5F1D	ATP Synthase F1 Subunit Delta	Protein Coding	2
7	ATP5F1B	ATP Synthase F1 Subunit Beta	Protein Coding	2
8	ATP5F1A	ATP Synthase F1 Subunit Alpha	Protein Coding	2
9	NDUFV2	NADH:Ubiquinone Oxidoreductase Core Subunit V2	Protein Coding	2
10	NDUFS8	NADH:Ubiquinone Oxidoreductase Core Subunit S8	Protein Coding	2
11	NDUFS4	NADH:Ubiquinone Oxidoreductase Subunit S4	Protein Coding	2
12	NDUFV1	NADH:Ubiquinone Oxidoreductase Core Subunit V1	Protein Coding	2
13	NDUFS3	NADH:Ubiquinone Oxidoreductase Core Subunit S3	Protein Coding	2
14	NDUFS2	NADH:Ubiquinone Oxidoreductase Core Subunit S2	Protein Coding	2
15	NDUFS1	NADH:Ubiquinone Oxidoreductase Core Subunit S1	Protein Coding	2
16	NDUFB9	NADH:Ubiquinone Oxidoreductase Subunit B9	Protein Coding	2
17	NDUFB8	NADH:Ubiquinone Oxidoreductase Subunit B8	Protein Coding	2
18	NDUFB7	NADH:Ubiquinone Oxidoreductase Subunit B7	Protein Coding	2
19	NDUFA2	NADH:Ubiquinone Oxidoreductase Subunit A2	Protein Coding	2
20	MT-ND6	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 6	Protein Coding	2
21	MT-ND5	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 5	Protein Coding	2
22	MT-ND4L	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 4L	Protein Coding	2
23	MT-ND4	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 4	Protein Coding	2
24	MT-ND2	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 2	Protein Coding	2
25	MT-ND1	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 1	Protein Coding	2
26	MT-ATP6	Mitochondrially Encoded ATP Synthase Membrane Subunit 6	Protein Coding	2
27	ATP5PD	ATP Synthase Peripheral Stalk Subunit D	Protein Coding	2
28	ATP5MF	ATP Synthase Membrane Subunit F	Protein Coding	2
29	NDUFA11	NADH:Ubiquinone Oxidoreductase Subunit A11	Protein Coding	2
30	ATP5MG	ATP Synthase Membrane Subunit G	Protein Coding	2
31	ATP5PO	ATP Synthase Peripheral Stalk Subunit OSCP	Protein Coding	2
32	ATP5MC3	ATP Synthase Membrane Subunit C Locus 3	Protein Coding	2
33	ATP5MC2	ATP Synthase Membrane Subunit C Locus 2	Protein Coding	2
34	ATP5MC1	ATP Synthase Membrane Subunit C Locus 1	Protein Coding	2
35	NDUFV3	NADH:Ubiquinone Oxidoreductase Subunit V3	Protein Coding	2
36	NDUFS6	NADH:Ubiquinone Oxidoreductase Subunit S6	Protein Coding	2
37	NDUFS5	NADH:Ubiquinone Oxidoreductase Subunit S5	Protein Coding	2
38	NDUFC2	NADH:Ubiquinone Oxidoreductase Subunit C2	Protein Coding	2
39	NDUFC1	NADH:Ubiquinone Oxidoreductase Subunit C1	Protein Coding	2
40	NDUFB10	NADH:Ubiquinone Oxidoreductase Subunit B10	Protein Coding	2
41	NDUFB6	NADH:Ubiquinone Oxidoreductase Subunit B6	Protein Coding	2
42	NDUFB5	NADH:Ubiquinone Oxidoreductase Subunit B5	Protein Coding	2
43	NDUFB4	NADH:Ubiquinone Oxidoreductase Subunit B4	Protein Coding	2
44	NDUFB2	NADH:Ubiquinone Oxidoreductase Subunit B2	Protein Coding	2
45	NDUFB1	NADH:Ubiquinone Oxidoreductase Subunit B1	Protein Coding	2
46	NDUFA10	NADH:Ubiquinone Oxidoreductase Subunit A10	Protein Coding	2
47	NDUFA9	NADH:Ubiquinone Oxidoreductase Subunit A9	Protein Coding	2
48	NDUFA8	NADH:Ubiquinone Oxidoreductase Subunit A8	Protein Coding	2
49	NDUFA7	NADH:Ubiquinone Oxidoreductase Subunit A7	Protein Coding	2
50	NDUFA6	NADH:Ubiquinone Oxidoreductase Subunit A6	Protein Coding	2
51	NDUFA5	NADH:Ubiquinone Oxidoreductase Subunit A5	Protein Coding	2
52	COXFA4	Cytochrome C Oxidase Associated Subunit FA4	Protein Coding	2
53	NDUFA3	NADH:Ubiquinone Oxidoreductase Subunit A3	Protein Coding	2
54	MT-ND3	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 3	Protein Coding	2
55	NDUFS7	NADH:Ubiquinone Oxidoreductase Core Subunit S7	Protein Coding	2
56	DMAC2L	Distal Membrane Arm Assembly Component 2 Like	Protein Coding	2
57	ATP5IF1	ATP Synthase Inhibitory Factor Subunit 1	Protein Coding	1
58	UQCRFS1	Ubiquinol-Cytochrome C Reductase, Rieske Iron-Sulfur Polypeptide 1	Protein Coding	1
59	UQCRC2	Ubiquinol-Cytochrome C Reductase Core Protein 2	Protein Coding	1
60	UQCRB	Ubiquinol-Cytochrome C Reductase Binding Protein	Protein Coding	1
61	UCP3	Uncoupling Protein 3	Protein Coding	1
62	UCP2	Uncoupling Protein 2	Protein Coding	1
63	UCP1	Uncoupling Protein 1	Protein Coding	1
64	SURF1	SURF1 Cytochrome C Oxidase Assembly Factor	Protein Coding	1
65	SDHD	Succinate Dehydrogenase Complex Subunit D	Protein Coding	1
66	SDHC	Succinate Dehydrogenase Complex Subunit C	Protein Coding	1
67	SDHB	Succinate Dehydrogenase Complex Iron Sulfur Subunit B	Protein Coding	1
68	SDHA	Succinate Dehydrogenase Complex Flavoprotein Subunit A	Protein Coding	1
69	SCO1	Synthesis Of Cytochrome C Oxidase 1	Protein Coding	1
70	MT-CYB	Mitochondrially Encoded Cytochrome B	Protein Coding	1
71	MT-CO3	Mitochondrially Encoded Cytochrome C Oxidase III	Protein Coding	1
72	MT-CO2	Mitochondrially Encoded Cytochrome C Oxidase II	Protein Coding	1
73	MT-CO1	Mitochondrially Encoded Cytochrome C Oxidase I	Protein Coding	1
74	SLC25A4	Solute Carrier Family 25 Member 4	Protein Coding	1
75	UQCRQ	Ubiquinol-Cytochrome C Reductase Complex III Subunit VII	Protein Coding	1
76	COX15	Cytochrome C Oxidase Assembly Factor COX15	Protein Coding	1
77	COX7C	Cytochrome C Oxidase Subunit 7C	Protein Coding	1
78	COX7B	Cytochrome C Oxidase Subunit 7B	Protein Coding	1
79	COX6B1	Cytochrome C Oxidase Subunit 6B1	Protein Coding	1
80	COX6A1	Cytochrome C Oxidase Subunit 6A1	Protein Coding	1
81	COX5B	Cytochrome C Oxidase Subunit 5B	Protein Coding	1
82	UQCR11	Ubiquinol-Cytochrome C Reductase, Complex III Subunit XI	Protein Coding	1
83	COX17	Cytochrome C Oxidase Copper Chaperone COX17	Protein Coding	1
84	SLC25A27	Solute Carrier Family 25 Member 27	Protein Coding	1
85	COX5A	Cytochrome C Oxidase Subunit 5A	Protein Coding	1
86	COX7A2L	Cytochrome C Oxidase Subunit 7A2 Like	Protein Coding	1
87	SLC25A14	Solute Carrier Family 25 Member 14	Protein Coding	1
88	COX11	Cytochrome C Oxidase Copper Chaperone COX11	Protein Coding	1
89	COX8A	Cytochrome C Oxidase Subunit 8A	Protein Coding	1
90	COX7A2	Cytochrome C Oxidase Subunit 7A2	Protein Coding	1
91	COX7A1	Cytochrome C Oxidase Subunit 7A1	Protein Coding	1
92	COX6C	Cytochrome C Oxidase Subunit 6C	Protein Coding	1
93	COX6A2	Cytochrome C Oxidase Subunit 6A2	Protein Coding	1
94	COX4I1	Cytochrome C Oxidase Subunit 4I1	Protein Coding	1
95	UQCRH	Ubiquinol-Cytochrome C Reductase Hinge Protein	Protein Coding	1
96	UQCRC1	Ubiquinol-Cytochrome C Reductase Core Protein 1	Protein Coding	1
97	NDUFA12	NADH:Ubiquinone Oxidoreductase Subunit A12	Protein Coding	1
98	ATP5F1C	ATP Synthase F1 Subunit Gamma	Protein Coding	1
99	NDUFB3	NADH:Ubiquinone Oxidoreductase Subunit B3	Protein Coding	1
100	NDUFA1	NADH:Ubiquinone Oxidoreductase Subunit A1	Protein Coding	1
101	MT-ATP8	Mitochondrially Encoded ATP Synthase Membrane Subunit 8	Protein Coding	1
102	UQCR10	Ubiquinol-Cytochrome C Reductase, Complex III Subunit X	Protein Coding	1
103	SLC25A6	Solute Carrier Family 25 Member 6	Protein Coding	1
104	SLC25A5	Solute Carrier Family 25 Member 5	Protein Coding	1
105	COXFA4L2	Cytochrome C Oxidase Hypoxia Associated Subunit FA4L2	Protein Coding	1
106	ATP6AP1	ATPase H+ Transporting Accessory Protein 1	Protein Coding	1
107	GZMB	Granzyme B	Protein Coding	1
108	ATP6AP2	ATPase H+ Transporting Accessory Protein 2	Protein Coding	1
109	MTCO3P12	MT-CO3 Pseudogene 12	Pseudogene	1

Disorders associated with Electron transport chain: OXPHOS system in mitochondria SuperPath

according to GeneCards Suite gene sharing

#	Disorder	Type	Genes	Score
1	Leigh syndrome, nuclear	Enrichment	ATP5PO, MT-ATP6, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFS4, NDUFS7, NDUFV1	16.00
2	Mitochondrial complex v deficiency, mitochondrial type 1	Enrichment	MT-ATP6, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5	16.00
3	Neuropathy, ataxia, and retinitis pigmentosa	Enrichment	MT-ATP6, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5	16.00
4	Striatonigral degeneration, infantile, mitochondrial	Enrichment	MT-ATP6, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5	16.00
5	Leigh syndrome, mitochondrial	Enrichment	MT-ATP6, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, MT-ND6	16.00
6	Camptodactyly of fingers	Enrichment	MT-ATP6, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5	16.00
7	Leber hereditary optic neuropathy, modifier of	Enrichment	MT-ATP6, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFS2	12.53
8	Mitochondrial disease	Enrichment	ATP5F1D, MT-ATP6, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFA6, NDUFC2, NDUFS1, NDUFS4	11.72
9	Leber optic atrophy and dystonia	Enrichment	MT-ND1, MT-ND3, MT-ND4, MT-ND5, MT-ND6	11.36
10	Mitochondrial complex i deficiency, nuclear type 1	Enrichment	MT-ND1, MT-ND2, MT-ND3, MT-ND4, NDUFA11, NDUFA6, NDUFB10, NDUFB9, NDUFS1, NDUFS2, NDUFS3, NDUFS4, NDUFS6, NDUFS7, NDUFS8, NDUFV1, NDUFV2	11.19
11	Cox deficiency, benign infantile mitochondrial myopathy	Enrichment	COX4I1, COX5A, COX6A2, COX6B1, COX8A, MT-CO1, MT-CO2, MT-CO3	10.96
12	Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes	Enrichment	MT-ATP6, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, MT-ND6, NDUFS1	10.96
13	Mitochondrial complex iv deficiency, nuclear type 1	Enrichment	COX4I1, COX6B1, COX8A, MT-CO1, MT-CO2, MT-CO3, NDUFV1, SCO1, SURF1	10.78
14	Mitochondrial myopathy, infantile, transient	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND5	10.77
15	Leber plus disease	Enrichment	MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFS2	10.76
16	Leigh disease	Enrichment	ATP5PO, MT-ATP6, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFS1, NDUFS4, NDUFS7, NDUFS8, NDUFV1	10.71
17	Mitochondrial myopathy with reversible cytochrome c oxidase deficiency	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND5	10.56
18	Hypertelorism	Enrichment	MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5	10.33
19	Isolated atp synthase deficiency	Enrichment	ATP5F1A, ATP5F1D, ATP5F1E, MT-ATP6, MT-ATP8	8.87
20	Isolated complex iii deficiency	Enrichment	MT-CYB, UQCRB, UQCRC2, UQCRFS1, UQCRQ	7.97
21	Retinitis pigmentosa	Enrichment	ATP5ME, MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5	7.12
22	Mitochondrial complex v deficiency, nuclear type 5	Enrichment	ATP5F1D, MT-ND5, NDUFV1	7.06
23	Carney triad	Enrichment	SDHA, SDHB, SDHC	6.36
24	Paraganglioma	Enrichment	SDHA, SDHB, SDHD	6.36
25	Familial colorectal cancer	Enrichment	MT-CO1, MT-CO2, MT-CYB, MT-ND4L	6.18
26	Pheochromocytoma/paraganglioma syndrome 1	Enrichment	SDHA, SDHB, SDHD	5.76
27	Mitochondrial complex ii deficiency, nuclear type 1	Enrichment	NDUFS8, SDHA, SDHD	5.76
28	Cardiomyopathy, infantile histiocytoid	Enrichment	MT-ATP6, MT-ATP8, MT-CYB	5.76
29	Mitochondrial complex ii deficiency	Enrichment	SDHA, SDHB, SDHD	5.76
30	Paraganglioma and gastric stromal sarcoma	Enrichment	SDHB, SDHC, SDHD	5.36
31	Gastrointestinal stromal tumor	Enrichment	SDHA, SDHB, SDHC	4.82
32	Hereditary paraganglioma-pheochromocytoma syndromes	Enrichment	SDHA, SDHB, SDHC, SDHD	4.75
33	Alzheimer disease mitochondrial	Enrichment	MT-ND1, MT-ND2	4.70
34	Cowden syndrome	Enrichment	SDHB, SDHC, SDHD	4.45
35	Optic nerve disease	Enrichment	MT-ATP6, MT-ATP8, MT-ND1	4.30
36	Rare mitochondrial non-syndromic sensorineural deafness	Enrichment	MT-CO1, MT-ND1, MT-ND4	4.30
37	Cardiomyopathy, infantile hypertrophic	Enrichment	MT-ATP6, MT-ATP8	4.23
38	Mitochondrial complex iv deficiency, nuclear type 6	Enrichment	COX15, SURF1	4.23
39	Periodic paralysis with later-onset distal motor neuropathy	Enrichment	MT-ATP6, MT-ATP8	4.23
40	Lactic acidosis	Enrichment	ATP5F1A, NDUFS4, UQCRFS1	4.04
41	Pheochromocytoma	Enrichment	SDHA, SDHB, SDHD	3.82
42	Pheochromocytoma/paraganglioma syndrome 4	Enrichment	SDHB, SDHD	3.76
43	Parkinson disease 6, autosomal recessive early-onset	Enrichment	MT-ND5, MT-ND6	3.53
44	Mitochondrial complex v deficiency, nuclear type 3	Enrichment	ATP5F1E, UQCRC2	3.46
45	Hereditary recurrent myoglobinuria	Enrichment	MT-CO1, MT-CO3	3.46
46	Cox deficiency, infantile mitochondrial myopathy	Enrichment	COX15, SCO1	3.24
47	Deafness, nonsyndromic sensorineural, mitochondrial	Enrichment	MT-CO1, MT-ND1	3.07
48	Sporadic pheochromocytoma/secreting paraganglioma	Enrichment	SDHB, SDHD	3.07
49	Tetralogy of fallot	Enrichment	MT-CO1, MT-CO2, MT-CO3	3.00
50	Optic atrophy plus syndrome	Enrichment	MT-ND1, MT-ND4, MT-ND6	3.00
51	Aortic valve disease 1	Enrichment	MT-ATP6, MT-ATP8	2.37
52	Ataxia and polyneuropathy, adult-onset	Enrichment	MT-ATP6	2.35
53	Congenital disorder of glycosylation, type iir	Enrichment	ATP6AP2	2.35
54	Mitochondrial complex i deficiency, mitochondrial type 1	Enrichment	MT-ND3	2.35
55	Myopathy, lactic acidosis, and sideroblastic anemia 3	Enrichment	MT-ATP6	2.35
56	Mitochondrial complex i deficiency, nuclear type 37	Enrichment	NDUFA8	2.35
57	Dystonia, early-onset, and/or spastic paraplegia	Enrichment	ATP5MC3	2.35
58	Parkinsonism with spasticity, x-linked	Enrichment	ATP6AP2	2.35
59	Mitochondrial complex i deficiency, nuclear type 9	Enrichment	NDUFS6	2.35
60	Mitochondrial complex i deficiency, nuclear type 22	Enrichment	NDUFA10	2.35
61	Mitochondrial complex i deficiency, nuclear type 8	Enrichment	NDUFS3	2.35
62	Mitochondrial complex i deficiency, nuclear type 26	Enrichment	NDUFA9	2.35
63	Intellectual developmental disorder, x-linked, syndromic, hedera type	Enrichment	ATP6AP2	2.35
64	Mitochondrial complex v deficiency, nuclear type 4b	Enrichment	ATP5F1A	2.35
65	Oncocytoma	Enrichment	MT-ND6	2.35
66	Combined oxidative phosphorylation deficiency 22	Enrichment	ATP5F1A	2.35
67	Mitochondrial complex i deficiency, nuclear type 24	Enrichment	NDUFB9	2.35
68	Mitochondrial complex i deficiency, nuclear type 33	Enrichment	NDUFA6	2.35
69	Mitochondrial complex i deficiency, nuclear type 6	Enrichment	NDUFS2	2.35
70	Mitochondrial complex i deficiency, nuclear type 32	Enrichment	NDUFB8	2.35
71	Mitochondrial complex v deficiency, nuclear type 4a	Enrichment	ATP5F1A	2.35
72	Mitochondrial complex iv deficiency, nuclear type 21	Enrichment	COXFA4	2.35
73	Hypermetabolism due to uncoupled mitochondrial oxidative phosphorylation 2	Enrichment	ATP5F1B	2.35
74	Mitochondrial complex i deficiency, nuclear type 39	Enrichment	NDUFB7	2.35
75	Leber-like hereditary optic neuropathy, autosomal recessive 2	Enrichment	NDUFS2	2.35
76	Progressive cavitating leukoencephalopathy	Enrichment	NDUFV2	2.35
77	Syndromic x-linked intellectual disability hedera type	Enrichment	ATP6AP2	2.35
78	Mitochondrial disorder due to a defect in assembly or maturation of the respiratory chain complexes	Enrichment	NDUFB7	2.35
79	Maternally-inherited spastic paraplegia	Enrichment	MT-ATP6	2.35
80	Rhabdomyosarcoma	Enrichment	SDHA, SDHC	2.19
81	Body mass index quantitative trait locus 11	Enrichment	MT-CYB, UCP2, UCP3	2.18
82	Linear skin defects with multiple congenital anomalies 2	Enrichment	COX7B	2.12
83	Myoglobinuria, recurrent	Enrichment	MT-CO1	2.12
84	Progressive external ophthalmoplegia with mitochondrial dna deletions, autosomal dominant 2	Enrichment	SLC25A4	2.12
85	Mitochondrial complex iii deficiency, nuclear type 3	Enrichment	UQCRB	2.12
86	Pheochromocytoma/paraganglioma syndrome 3	Enrichment	SDHC	2.12
87	Pheochromocytoma/paraganglioma syndrome 5	Enrichment	SDHA	2.12
88	Mitochondrial dna depletion syndrome 12b , autosomal recessive	Enrichment	SLC25A4	2.12
89	Mitochondrial complex iv deficiency, nuclear type 18	Enrichment	COX6A2	2.12
90	Mitochondrial complex i deficiency, nuclear type 23	Enrichment	NDUFA12	2.12
91	Mitochondrial complex i deficiency, nuclear type 25	Enrichment	NDUFB3	2.12
92	Mitochondrial complex iv deficiency, nuclear type 23	Enrichment	COX11	2.12
93	Body mass index quantitative trait locus 4	Enrichment	UCP2	2.12
94	Mitochondrial dna depletion syndrome 12a , autosomal dominant	Enrichment	SLC25A4	2.12
95	Cardiomyopathy, dilated, 1gg	Enrichment	SDHA	2.12
96	Mitochondrial complex iii deficiency, nuclear type 10	Enrichment	UQCRFS1	2.12
97	Mitochondrial metabolism disease	Enrichment	SLC25A4	2.12
98	Neurodegeneration with ataxia and late-onset optic atrophy	Enrichment	SDHA	2.12
99	Mitochondrial complex iv deficiency, nuclear type 7	Enrichment	COX6B1	2.12
100	Mitochondrial complex iv deficiency, nuclear type 20	Enrichment	COX5A	2.12
101	Mitochondrial complex iii deficiency, nuclear type 11	Enrichment	UQCRH	2.12
102	Mitochondrial complex iv deficiency, nuclear type 16	Enrichment	COX4I1	2.12
103	Mitochondrial complex iv deficiency, nuclear type 15	Enrichment	COX8A	2.12
104	Charcot-marie-tooth disease, recessive intermediate d	Enrichment	COX6A1	2.12
105	Carcinoid syndrome	Enrichment	SDHD	2.12
106	Autosomal dominant spastic ataxia	Enrichment	MT-CO3	2.12
107	Hyperinsulinism due to ucp2 deficiency	Enrichment	UCP2	2.12
108	Charcot-marie-tooth disease, demyelinating, type 1a	Enrichment	MT-ATP6	2.05
109	Deafness, sensorineural, autosomal-mitochondrial type	Enrichment	MT-ND1	2.05
110	L-2-hydroxyglutaric aciduria	Enrichment	DMAC2L	2.05
111	Leukoencephalopathy, cystic, without megalencephaly	Enrichment	NDUFA2	2.05
112	Mitochondrial complex i deficiency, nuclear type 14	Enrichment	NDUFA11	2.05
113	Mitochondrial complex i deficiency, nuclear type 36	Enrichment	NDUFC2	2.05
114	Charcot-marie-tooth disease type 1a	Enrichment	MT-ATP6	2.05
115	Nuclear type mitochondrial complex i deficiency	Enrichment	NDUFV1	2.05
116	Mitochondrial complex i deficiency, nuclear type 13	Enrichment	NDUFA2	2.05
117	Mitochondrial complex i deficiency, nuclear type 7	Enrichment	NDUFV2	2.05
118	Dystonia	Enrichment	MT-ND1, MT-ND6	1.97
119	Mitochondrial complex iii deficiency, nuclear type 1	Enrichment	NDUFS7	1.87
120	Mitochondrial myopathy, episodic, with or without optic atrophy and reversible leukoencephalopathy	Enrichment	MT-ND3	1.87
121	Mitochondrial complex iv deficiency, nuclear type 5	Enrichment	ATP5F1A	1.87
122	Retinitis pigmentosa 40	Enrichment	ATP5ME	1.87
123	Mitochondrial complex v deficiency, nuclear type 7	Enrichment	ATP5PO	1.87
124	Mitochondrial complex v deficiency, nuclear type 1	Enrichment	NDUFS1	1.87
125	Mitochondrial complex i deficiency, nuclear type 35	Enrichment	NDUFB10	1.87
126	Gonadal dysgenesis	Enrichment	MT-ATP6	1.87
127	Polymyoclonus, infantile	Enrichment	SDHA	1.82
128	Mitochondrial complex i deficiency, nuclear type 12	Enrichment	NDUFA1	1.82
129	Charcot-marie-tooth disease type 4k	Enrichment	SURF1	1.82
130	Charcot-marie-tooth disease, demyelinating, type 4k	Enrichment	SURF1	1.82
131	Mitochondrial complex iv deficiency, nuclear type 4	Enrichment	SCO1	1.82
132	Parkinsonism with polyneuropathy	Enrichment	UQCRC1	1.82
133	Familial infantile bilateral striatal necrosis	Enrichment	MT-ATP6	1.75
134	Inherited cancer-predisposing syndrome	Enrichment	SDHA, SDHB, SDHC, SDHD	1.73
135	Albinism, oculocutaneous, type ii	Enrichment	ATP5ME	1.65
136	Keratoderma, palmoplantar, with deafness	Enrichment	MT-CO1	1.64
137	Sengers syndrome	Enrichment	SLC25A4	1.64
138	Linear skin defects with multiple congenital anomalies 1	Enrichment	COX7B	1.64
139	Mitochondrial complex iv deficiency, nuclear type 2	Enrichment	SDHB	1.64
140	Mitochondrial complex iii deficiency, nuclear type 2	Enrichment	SDHD	1.64
141	Brain cancer	Enrichment	SDHA	1.64
142	Myelodysplastic syndrome with ring sideroblasts	Enrichment	MT-CO1	1.64
143	Renal cell carcinoma	Enrichment	SDHB	1.64
144	Propionic acidemia	Enrichment	UQCRFS1	1.52
145	Leptin deficiency or dysfunction	Enrichment	UCP3	1.52
146	Pilocytic astrocytoma	Enrichment	SDHA	1.52
147	Parkinsonism	Enrichment	MT-ND6	1.51
148	Immunodeficiency 47	Enrichment	ATP6AP1	1.45
149	Epilepsy	Enrichment	MT-CO3, MT-CYB	1.42
150	Von hippel-lindau syndrome	Enrichment	SDHB	1.42
151	Kearns-sayre syndrome	Enrichment	MT-ATP8	1.42
152	Mitochondrial complex iii deficiency, nuclear type 4	Enrichment	UQCRQ	1.42
153	Hypertrophic cardiomyopathy	Enrichment	SLC25A4, UQCRC1	1.37
154	Hereditary breast carcinoma	Enrichment	MT-CYB, SDHA	1.35
155	Progressive external ophthalmoplegia with mitochondrial dna deletions, autosomal dominant 1	Enrichment	SLC25A4	1.35
156	Migraine with or without aura 1	Enrichment	MT-ND1	1.32
157	Myoclonic epilepsy associated with ragged-red fibers	Enrichment	MT-ND5	1.28
158	Restrictive cardiomyopathy	Enrichment	MT-ND1	1.28
159	Alzheimer's disease	Enrichment	MT-ND1	1.24
160	Hydrocephalus, congenital, 1	Enrichment	MT-ND1	1.16
161	Alzheimer disease, familial, 1	Enrichment	MT-ND1	1.13
162	Sudden infant death syndrome	Enrichment	MT-ND1	1.13
163	Mitochondrial encephalomyopathy	Enrichment	MT-CYB	1.13
164	Mitochondrial myopathy	Enrichment	SLC25A4	1.09
165	Parkinson's disease	Enrichment	MT-ND1	1.09
166	Attention deficit-hyperactivity disorder	Enrichment	MT-ND1	1.02
167	Parkinson disease, late-onset	Enrichment	MT-ND1	0.99
168	Myopia	Enrichment	SLC25A4	0.96
169	Auditory neuropathy	Enrichment	MT-ND6	0.96
170	Breast cancer	Enrichment	MT-CYB, SDHA	0.95
171	Hereditary retinal dystrophy	Enrichment	ATP5ME, MT-ND4, MT-ND6	0.85
172	Fundus dystrophy	Enrichment	ATP5ME, MT-ND4, MT-ND6	0.85
173	Colorectal cancer	Enrichment	MT-CO1, MT-ND1	0.84
174	Non-syndromic x-linked intellectual disability	Enrichment	ATP6AP1	0.82
175	Charcot-marie-tooth disease	Enrichment	MT-ATP6	0.77
176	Peripheral nervous system disease	Enrichment	COX6A1	0.66
177	Neuropathy	Enrichment	COX6A1	0.66
178	Rare genetic deafness	Enrichment	MT-ND1	0.52
179	Familial isolated dilated cardiomyopathy	Enrichment	SDHA	0.47
180	Microcephaly	Enrichment	ATP5PO	0.37
181	Ovarian cancer	Enrichment	MT-CYB	0.27

¹ WikiPathways

² PubChem

³ R&D Systems

⁴ Reactome

⁵ GeneGo (Thomson Reuters)

⁶ QIAGEN

⁷ PharmGKB

⁸ Cell Signaling Technology

⁹ Sino Biological

¹⁰ Tocris

¹¹ MalaCards

Electron transport chain: OXPHOS system in mitochondria

Pathway Network for Electron transport chain: OXPHOS system in mitochondria

Member Pathways for Electron transport chain: OXPHOS system in mitochondria

Pathways in the Electron transport chain: OXPHOS system in mitochondria SuperPath

Associated Genes for Electron transport chain: OXPHOS system in mitochondria

Associated Disorders for Electron transport chain: OXPHOS system in mitochondria

Disorders associated with Electron transport chain: OXPHOS system in mitochondria SuperPath

STRING Interaction Network for Electron transport chain: OXPHOS system in mitochondria

Sources for Electron transport chain: OXPHOS system in mitochondria