Respiratory electron transport

Pathway network for the Respiratory electron transport SuperPath

Sources:

Reactome

Pathways in the Respiratory electron transport SuperPath

#	Name	Source	Genes
1	Respiratory electron transport	Reactome	ACAD9 BCS1L CMC1 COA1 COA3 COA5 COQ10A COQ10B COX11 COX14 COX15 COX16 COX17 COX18 COX19 COX20 COX4I1 COX4I2 COX5A COX5B COX6A1 COX6A2 COX6B1 COX6B2 COX6C COX7A1 COX7A2 COX7A2L COX7B COX7C COX8A COX8C COXFA4 CYC1 DMAC1 DMAC2 ETFA ETFB ETFDH FOXRED1 FXN GOT1 GOT2 HCCS HIGD1A HIGD1C HIGD2A HSCB HSPA9 ISCU LETM1 LYRM2 LYRM4 LYRM7 MDH1 MDH2 MT-CO1 MT-CO2 MT-CO3 MT-CYB MT-ND1 MT-ND2 MT-ND3 MT-ND4 MT-ND5 MT-ND6 NDUFA1 NDUFA10 NDUFA11 NDUFA12 NDUFA13 NDUFA2 NDUFA3 NDUFA5 NDUFA6 NDUFA7 NDUFA8 NDUFA9 NDUFAB1 NDUFAF1 NDUFAF2 NDUFAF3 NDUFAF4 NDUFAF5 NDUFAF6 NDUFAF7 NDUFAF8 NDUFB1 NDUFB10 NDUFB11 NDUFB2 NDUFB3 NDUFB4 NDUFB5 NDUFB6 NDUFB7 NDUFB8 NDUFB9 NDUFC1 NDUFC2 NDUFS1 NDUFS2 NDUFS3 NDUFS4 NDUFS5 NDUFS6 NDUFS7 NDUFS8 NDUFV1 NDUFV2 NDUFV3 NFS1 NUBPL OXA1L PET100 PET117 PNKD PYURF RAB5IF SCO1 SCO2 SDHA SDHB SDHC SDHD SFXN4 SLC25A11 SLC25A12 SLC25A13 SLC25A18 SLC25A22 SMIM20 SURF1 TACO1 TIMM21 TIMMDC1 TMEM126A TMEM126B TMEM177 TMEM186 TMEM223 TRAP1 TTC19 UQCC1 UQCC2 UQCC3 UQCC5 UQCC6 UQCR10 UQCR11 UQCRB UQCRC1 UQCRC2 UQCRFS1 UQCRH UQCRHL UQCRQ (see all 157) (see less)
2	Aerobic respiration and respiratory electron transport	Reactome	ACAD9 ACAT1 ACO2 ADHFE1 ARMC8 ATP5F1A ATP5F1B ATP5F1C ATP5F1D ATP5F1E ATP5MC1 ATP5MC2 ATP5MC3 ATP5ME ATP5MF ATP5MG ATP5MJ ATP5MK ATP5PB ATP5PD ATP5PF ATP5PO BCS1L CMC1 COA1 COA3 COA5 COQ10A COQ10B COX11 COX14 COX15 COX16 COX17 COX18 COX19 COX20 COX4I1 COX4I2 COX5A COX5B COX6A1 COX6A2 COX6B1 COX6B2 COX6C COX7A1 COX7A2 COX7A2L COX7B COX7C COX8A COX8C COXFA4 CS CSKMT CYC1 D2HGDH DLAT DLD DLST DMAC1 DMAC2 DMAC2L ETFA ETFB ETFDH FAHD1 FH FOXRED1 FXN GID4 GID8 GLO1 GOT1 GOT2 GPT GSTZ1 HAGH HCCS HIGD1A HIGD1C HIGD2A HSCB HSPA9 IDH2 IDH3A IDH3B IDH3G ISCA1 ISCA2 ISCU KGD4 L2HGDH LDHA LDHAL6A LDHAL6B LDHB LDHC LETM1 LYRM2 LYRM4 LYRM7 MAEA MDH1 MDH2 ME1 ME2 ME3 MKLN1 MPC1 MPC1L MPC2 MT-ATP6 MT-ATP8 MT-CO1 MT-CO2 MT-CO3 MT-CYB MT-ND1 MT-ND2 MT-ND3 MT-ND4 MT-ND5 MT-ND6 NDUFA1 NDUFA10 NDUFA11 NDUFA12 NDUFA13 NDUFA2 NDUFA3 NDUFA5 NDUFA6 NDUFA7 NDUFA8 NDUFA9 NDUFAB1 NDUFAF1 NDUFAF2 NDUFAF3 NDUFAF4 NDUFAF5 NDUFAF6 NDUFAF7 NDUFAF8 NDUFB1 NDUFB10 NDUFB11 NDUFB2 NDUFB3 NDUFB4 NDUFB5 NDUFB6 NDUFB7 NDUFB8 NDUFB9 NDUFC1 NDUFC2 NDUFS1 NDUFS2 NDUFS3 NDUFS4 NDUFS5 NDUFS6 NDUFS7 NDUFS8 NDUFV1 NDUFV2 NDUFV3 NEK1 NFS1 NNT NUBPL OGDH OXA1L PC PDHA1 PDHA2 PDHB PDHX PDK1 PDK2 PDK3 PDK4 PDP1 PDP2 PDPR PET100 PET117 PGAM5 PKLR PKM PM20D1 PNKD PYURF RAB5IF RANBP9 RMND5A RMND5B RPS27A SCO1 SCO2 SDHA SDHAF1 SDHAF2 SDHAF3 SDHAF4 SDHB SDHC SDHD SFXN4 SIRT3 SIRT4 SLC25A11 SLC25A12 SLC25A13 SLC25A14 SLC25A18 SLC25A22 SLC25A27 SMIM20 SUCLA2 SUCLG1 SUCLG2 SURF1 TACO1 TIMM21 TIMMDC1 TMEM126A TMEM126B TMEM177 TMEM186 TMEM223 TRAP1 TTC19 UBA52 UBB UBC UCP1 UCP2 UCP3 UQCC1 UQCC2 UQCC3 UQCC5 UQCC6 UQCR10 UQCR11 UQCRB UQCRC1 UQCRC2 UQCRFS1 UQCRH UQCRHL UQCRQ VDAC1 WDR26 (see all 258) (see less)
3	Malate-aspartate shuttle	Reactome	GOT1 GOT2 MDH1 MDH2 SLC25A11 SLC25A12 SLC25A13 SLC25A18 SLC25A22 (see all 9) (see less)

Gene overlap in member pathways for Respiratory electron transport SuperPath

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

#	Symbol	Description	Category	# Related Pathways
1	SLC25A13	Solute Carrier Family 25 Member 13	Protein Coding	3
2	GOT1	Glutamic-Oxaloacetic Transaminase 1	Protein Coding	3
3	GOT2	Glutamic-Oxaloacetic Transaminase 2	Protein Coding	3
4	MDH1	Malate Dehydrogenase 1	Protein Coding	3
5	MDH2	Malate Dehydrogenase 2	Protein Coding	3
6	SLC25A22	Solute Carrier Family 25 Member 22	Protein Coding	3
7	SLC25A18	Solute Carrier Family 25 Member 18	Protein Coding	3
8	SLC25A11	Solute Carrier Family 25 Member 11	Protein Coding	3
9	SLC25A12	Solute Carrier Family 25 Member 12	Protein Coding	3
10	PET100	PET100 Cytochrome C Oxidase Chaperone	Protein Coding	2
11	PET117	PET117 Cytochrome C Oxidase Chaperone	Protein Coding	2
12	COX17	Cytochrome C Oxidase Copper Chaperone COX17	Protein Coding	2
13	PYURF	PIGY Upstream Open Reading Frame	Protein Coding	2
14	TRAP1	TNF Receptor Associated Protein 1	Protein Coding	2
15	UQCR11	Ubiquinol-Cytochrome C Reductase, Complex III Subunit XI	Protein Coding	2
16	COX20	Cytochrome C Oxidase Assembly Factor COX20	Protein Coding	2
17	SFXN4	Sideroflexin 4	Protein Coding	2
18	COX6B2	Cytochrome C Oxidase Subunit 6B2	Protein Coding	2
19	NDUFA11	NADH:Ubiquinone Oxidoreductase Subunit A11	Protein Coding	2
20	COX4I1	Cytochrome C Oxidase Subunit 4I1	Protein Coding	2
21	COX5B	Cytochrome C Oxidase Subunit 5B	Protein Coding	2
22	COX6A1	Cytochrome C Oxidase Subunit 6A1	Protein Coding	2
23	COX6A2	Cytochrome C Oxidase Subunit 6A2	Protein Coding	2
24	COX6B1	Cytochrome C Oxidase Subunit 6B1	Protein Coding	2
25	COX6C	Cytochrome C Oxidase Subunit 6C	Protein Coding	2
26	COX7A1	Cytochrome C Oxidase Subunit 7A1	Protein Coding	2
27	COX7A2	Cytochrome C Oxidase Subunit 7A2	Protein Coding	2
28	COX7B	Cytochrome C Oxidase Subunit 7B	Protein Coding	2
29	COX7C	Cytochrome C Oxidase Subunit 7C	Protein Coding	2
30	COX8A	Cytochrome C Oxidase Subunit 8A	Protein Coding	2
31	COX11	Cytochrome C Oxidase Copper Chaperone COX11	Protein Coding	2
32	COX15	Cytochrome C Oxidase Assembly Factor COX15	Protein Coding	2
33	NDUFAF6	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 6	Protein Coding	2
34	HSCB	HscB Mitochondrial Iron-Sulfur Cluster Cochaperone	Protein Coding	2
35	CMC1	C-X9-C Motif Containing 1	Protein Coding	2
36	CYC1	Cytochrome C1	Protein Coding	2
37	HIGD2A	HIG1 Hypoxia Inducible Domain Family Member 2A	Protein Coding	2
38	ETFA	Electron Transfer Flavoprotein Subunit Alpha	Protein Coding	2
39	ETFB	Electron Transfer Flavoprotein Subunit Beta	Protein Coding	2
40	ETFDH	Electron Transfer Flavoprotein Dehydrogenase	Protein Coding	2
41	ISCU	Iron-Sulfur Cluster Assembly Enzyme	Protein Coding	2
42	FXN	Frataxin	Protein Coding	2
43	TMEM186	Transmembrane Protein 186	Protein Coding	2
44	NDUFAF3	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 3	Protein Coding	2
45	PNKD	PNKD Metallo-Beta-Lactamase Domain Containing	Protein Coding	2
46	HIGD1A	HIG1 Hypoxia Inducible Domain Family Member 1A	Protein Coding	2
47	UQCRQ	Ubiquinol-Cytochrome C Reductase Complex III Subunit VII	Protein Coding	2
48	NDUFAF8	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 8	Protein Coding	2
49	COX18	Cytochrome C Oxidase Assembly Factor COX18	Protein Coding	2
50	COA3	Cytochrome C Oxidase Assembly Factor 3	Protein Coding	2
51	ACAD9	Acyl-CoA Dehydrogenase Family Member 9	Protein Coding	2
52	NDUFAF4	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 4	Protein Coding	2
53	TIMM21	Translocase Of Inner Mitochondrial Membrane 21	Protein Coding	2
54	UQCR10	Ubiquinol-Cytochrome C Reductase, Complex III Subunit X	Protein Coding	2
55	HCCS	Holocytochrome C Synthase	Protein Coding	2
56	HSPA9	Heat Shock Protein Family A (Hsp70) Member 9	Protein Coding	2
57	COX8C	Cytochrome C Oxidase Subunit 8C	Protein Coding	2
58	NDUFS7	NADH:Ubiquinone Oxidoreductase Core Subunit S7	Protein Coding	2
59	SMIM20	Small Integral Membrane Protein 20	Protein Coding	2
60	LETM1	Leucine Zipper And EF-Hand Containing Transmembrane Protein 1	Protein Coding	2
61	UQCRHL	Ubiquinol-Cytochrome C Reductase Hinge Protein Like	Protein Coding	2
62	UQCC5	Ubiquinol-Cytochrome C Reductase Complex Assembly Factor 5	Protein Coding	2
63	MT-CO1	Mitochondrially Encoded Cytochrome C Oxidase I	Protein Coding	2
64	MT-CO2	Mitochondrially Encoded Cytochrome C Oxidase II	Protein Coding	2
65	MT-CO3	Mitochondrially Encoded Cytochrome C Oxidase III	Protein Coding	2
66	MT-CYB	Mitochondrially Encoded Cytochrome B	Protein Coding	2
67	MT-ND1	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 1	Protein Coding	2
68	MT-ND2	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 2	Protein Coding	2
69	MT-ND3	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 3	Protein Coding	2
70	MT-ND4	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 4	Protein Coding	2
71	MT-ND5	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 5	Protein Coding	2
72	MT-ND6	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 6	Protein Coding	2
73	NDUFA1	NADH:Ubiquinone Oxidoreductase Subunit A1	Protein Coding	2
74	NDUFA2	NADH:Ubiquinone Oxidoreductase Subunit A2	Protein Coding	2
75	NDUFA3	NADH:Ubiquinone Oxidoreductase Subunit A3	Protein Coding	2
76	COXFA4	Cytochrome C Oxidase Associated Subunit FA4	Protein Coding	2
77	NDUFA5	NADH:Ubiquinone Oxidoreductase Subunit A5	Protein Coding	2
78	NDUFA6	NADH:Ubiquinone Oxidoreductase Subunit A6	Protein Coding	2
79	NDUFA7	NADH:Ubiquinone Oxidoreductase Subunit A7	Protein Coding	2
80	NDUFA8	NADH:Ubiquinone Oxidoreductase Subunit A8	Protein Coding	2
81	NDUFA9	NADH:Ubiquinone Oxidoreductase Subunit A9	Protein Coding	2
82	NDUFA10	NADH:Ubiquinone Oxidoreductase Subunit A10	Protein Coding	2
83	NDUFAB1	NADH:Ubiquinone Oxidoreductase Subunit AB1	Protein Coding	2
84	NDUFB1	NADH:Ubiquinone Oxidoreductase Subunit B1	Protein Coding	2
85	NDUFB2	NADH:Ubiquinone Oxidoreductase Subunit B2	Protein Coding	2
86	NDUFB3	NADH:Ubiquinone Oxidoreductase Subunit B3	Protein Coding	2
87	NDUFB4	NADH:Ubiquinone Oxidoreductase Subunit B4	Protein Coding	2
88	NDUFB5	NADH:Ubiquinone Oxidoreductase Subunit B5	Protein Coding	2
89	NDUFB6	NADH:Ubiquinone Oxidoreductase Subunit B6	Protein Coding	2
90	NDUFB7	NADH:Ubiquinone Oxidoreductase Subunit B7	Protein Coding	2
91	NDUFB8	NADH:Ubiquinone Oxidoreductase Subunit B8	Protein Coding	2
92	NDUFB9	NADH:Ubiquinone Oxidoreductase Subunit B9	Protein Coding	2
93	NDUFB10	NADH:Ubiquinone Oxidoreductase Subunit B10	Protein Coding	2
94	NDUFC1	NADH:Ubiquinone Oxidoreductase Subunit C1	Protein Coding	2
95	NDUFC2	NADH:Ubiquinone Oxidoreductase Subunit C2	Protein Coding	2
96	NDUFS1	NADH:Ubiquinone Oxidoreductase Core Subunit S1	Protein Coding	2
97	NDUFS2	NADH:Ubiquinone Oxidoreductase Core Subunit S2	Protein Coding	2
98	NDUFS3	NADH:Ubiquinone Oxidoreductase Core Subunit S3	Protein Coding	2
99	NDUFV1	NADH:Ubiquinone Oxidoreductase Core Subunit V1	Protein Coding	2
100	NDUFS4	NADH:Ubiquinone Oxidoreductase Subunit S4	Protein Coding	2
101	NDUFS5	NADH:Ubiquinone Oxidoreductase Subunit S5	Protein Coding	2
102	NDUFS6	NADH:Ubiquinone Oxidoreductase Subunit S6	Protein Coding	2
103	NDUFS8	NADH:Ubiquinone Oxidoreductase Core Subunit S8	Protein Coding	2
104	NDUFV2	NADH:Ubiquinone Oxidoreductase Core Subunit V2	Protein Coding	2
105	NDUFV3	NADH:Ubiquinone Oxidoreductase Subunit V3	Protein Coding	2
106	COA5	Cytochrome C Oxidase Assembly Factor 5	Protein Coding	2
107	OXA1L	OXA1L Mitochondrial Inner Membrane Insertase	Protein Coding	2
108	NDUFA13	NADH:Ubiquinone Oxidoreductase Subunit A13	Protein Coding	2
109	NDUFAF1	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 1	Protein Coding	2
110	TACO1	Translational Activator Of Cytochrome C Oxidase I	Protein Coding	2
111	COX16	Cytochrome C Oxidase Assembly Factor COX16	Protein Coding	2
112	TIMMDC1	Translocase Of Inner Mitochondrial Membrane Domain Containing 1	Protein Coding	2
113	NDUFB11	NADH:Ubiquinone Oxidoreductase Subunit B11	Protein Coding	2
114	TTC19	Tetratricopeptide Repeat Domain 19	Protein Coding	2
115	DMAC2	Distal Membrane Arm Assembly Component 2	Protein Coding	2
116	UQCC1	Ubiquinol-Cytochrome C Reductase Complex Assembly Factor 1	Protein Coding	2
117	NDUFAF7	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 7	Protein Coding	2
118	FOXRED1	FAD Dependent Oxidoreductase Domain Containing 1	Protein Coding	2
119	COA1	Cytochrome C Oxidase Assembly Factor 1	Protein Coding	2
120	TMEM126B	Transmembrane Protein 126B	Protein Coding	2
121	NDUFA12	NADH:Ubiquinone Oxidoreductase Subunit A12	Protein Coding	2
122	RAB5IF	RAB5 Interacting Factor	Protein Coding	2
123	LYRM4	LYR Motif Containing 4	Protein Coding	2
124	LYRM2	LYR Motif Containing 2	Protein Coding	2
125	HIGD1C	HIG1 Hypoxia Inducible Domain Family Member 1C	Protein Coding	2
126	BCS1L	BCS1 Ubiquinol-Cytochrome C Reductase Complex Chaperone	Protein Coding	2
127	SCO1	Synthesis Of Cytochrome C Oxidase 1	Protein Coding	2
128	SDHA	Succinate Dehydrogenase Complex Flavoprotein Subunit A	Protein Coding	2
129	SDHB	Succinate Dehydrogenase Complex Iron Sulfur Subunit B	Protein Coding	2
130	SDHC	Succinate Dehydrogenase Complex Subunit C	Protein Coding	2
131	SDHD	Succinate Dehydrogenase Complex Subunit D	Protein Coding	2
132	SURF1	SURF1 Cytochrome C Oxidase Assembly Factor	Protein Coding	2
133	UQCC6	Ubiquinol-Cytochrome C Reductase Complex Assembly Factor 6	Protein Coding	2
134	UQCRB	Ubiquinol-Cytochrome C Reductase Binding Protein	Protein Coding	2
135	UQCRC1	Ubiquinol-Cytochrome C Reductase Core Protein 1	Protein Coding	2
136	UQCRC2	Ubiquinol-Cytochrome C Reductase Core Protein 2	Protein Coding	2
137	UQCRFS1	Ubiquinol-Cytochrome C Reductase, Rieske Iron-Sulfur Polypeptide 1	Protein Coding	2
138	UQCRH	Ubiquinol-Cytochrome C Reductase Hinge Protein	Protein Coding	2
139	TMEM223	Transmembrane Protein 223	Protein Coding	2
140	UQCC3	Ubiquinol-Cytochrome C Reductase Complex Assembly Factor 3	Protein Coding	2
141	NDUFAF5	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 5	Protein Coding	2
142	COQ10B	Coenzyme Q10B	Protein Coding	2
143	NUBPL	NUBP Iron-Sulfur Cluster Assembly Factor, Mitochondrial	Protein Coding	2
144	TMEM177	Transmembrane Protein 177	Protein Coding	2
145	TMEM126A	Transmembrane Protein 126A	Protein Coding	2
146	UQCC2	Ubiquinol-Cytochrome C Reductase Complex Assembly Factor 2	Protein Coding	2
147	COX4I2	Cytochrome C Oxidase Subunit 4I2	Protein Coding	2
148	COX14	Cytochrome C Oxidase Assembly Factor COX14	Protein Coding	2
149	NFS1	NFS1 Cysteine Desulfurase	Protein Coding	2
150	LYRM7	LYR Motif Containing 7	Protein Coding	2
151	COX19	Cytochrome C Oxidase Assembly Factor COX19	Protein Coding	2
152	DMAC1	Distal Membrane Arm Assembly Component 1	Protein Coding	2
153	COX7A2L	Cytochrome C Oxidase Subunit 7A2 Like	Protein Coding	2
154	NDUFAF2	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 2	Protein Coding	2
155	COQ10A	Coenzyme Q10A	Protein Coding	2
156	COX5A	Cytochrome C Oxidase Subunit 5A	Protein Coding	2
157	SCO2	Synthesis Of Cytochrome C Oxidase 2	Protein Coding	2
158	RANBP9	RAN Binding Protein 9	Protein Coding	1
159	MAEA	Macrophage Erythroblast Attacher, E3 Ubiquitin Ligase	Protein Coding	1
160	ATP5PD	ATP Synthase Peripheral Stalk Subunit D	Protein Coding	1
161	ATP5MG	ATP Synthase Membrane Subunit G	Protein Coding	1
162	ME3	Malic Enzyme 3	Protein Coding	1
163	ISCA2	Iron-Sulfur Cluster Assembly 2	Protein Coding	1
164	SDHAF4	Succinate Dehydrogenase Complex Assembly Factor 4	Protein Coding	1
165	ADHFE1	Alcohol Dehydrogenase Iron Containing 1	Protein Coding	1
166	CS	Citrate Synthase	Protein Coding	1
167	PM20D1	Peptidase M20 Domain Containing 1	Protein Coding	1
168	LDHAL6A	Lactate Dehydrogenase A Like 6A	Protein Coding	1
169	DLAT	Dihydrolipoamide S-Acetyltransferase	Protein Coding	1
170	DLD	Dihydrolipoamide Dehydrogenase	Protein Coding	1
171	DLST	Dihydrolipoamide S-Succinyltransferase	Protein Coding	1
172	PGAM5	PGAM Family Member 5, Mitochondrial Serine/Threonine Protein Phosphatase	Protein Coding	1
173	FH	Fumarate Hydratase	Protein Coding	1
174	SIRT4	Sirtuin 4	Protein Coding	1
175	SIRT3	Sirtuin 3	Protein Coding	1
176	NNT	Nicotinamide Nucleotide Transhydrogenase	Protein Coding	1
177	ARMC8	Armadillo Repeat Containing 8	Protein Coding	1
178	MPC2	Mitochondrial Pyruvate Carrier 2	Protein Coding	1
179	DMAC2L	Distal Membrane Arm Assembly Component 2 Like	Protein Coding	1
180	GLO1	Glyoxalase I	Protein Coding	1
181	GPT	Glutamic--Pyruvic Transaminase	Protein Coding	1
182	GSTZ1	Glutathione S-Transferase Zeta 1	Protein Coding	1
183	HAGH	Hydroxyacylglutathione Hydrolase	Protein Coding	1
184	IDH2	Isocitrate Dehydrogenase (NADP(+)) 2	Protein Coding	1
185	IDH3A	Isocitrate Dehydrogenase (NAD(+)) 3 Catalytic Subunit Alpha	Protein Coding	1
186	IDH3B	Isocitrate Dehydrogenase (NAD(+)) 3 Non-Catalytic Subunit Beta	Protein Coding	1
187	IDH3G	Isocitrate Dehydrogenase (NAD(+)) 3 Non-Catalytic Subunit Gamma	Protein Coding	1
188	MPC1L	Mitochondrial Pyruvate Carrier 1 Like	Protein Coding	1
189	ACAT1	Acetyl-CoA Acetyltransferase 1	Protein Coding	1
190	LDHA	Lactate Dehydrogenase A	Protein Coding	1
191	LDHB	Lactate Dehydrogenase B	Protein Coding	1
192	LDHC	Lactate Dehydrogenase C	Protein Coding	1
193	ME1	Malic Enzyme 1	Protein Coding	1
194	ME2	Malic Enzyme 2	Protein Coding	1
195	MKLN1	Muskelin 1	Protein Coding	1
196	MT-ATP6	Mitochondrially Encoded ATP Synthase Membrane Subunit 6	Protein Coding	1
197	MT-ATP8	Mitochondrially Encoded ATP Synthase Membrane Subunit 8	Protein Coding	1
198	NEK1	NIMA Related Kinase 1	Protein Coding	1
199	OGDH	Oxoglutarate Dehydrogenase	Protein Coding	1
200	ATP5F1A	ATP Synthase F1 Subunit Alpha	Protein Coding	1
201	ACO2	Aconitase 2	Protein Coding	1
202	ATP5F1B	ATP Synthase F1 Subunit Beta	Protein Coding	1
203	ATP5F1C	ATP Synthase F1 Subunit Gamma	Protein Coding	1
204	PC	Pyruvate Carboxylase	Protein Coding	1
205	ATP5F1D	ATP Synthase F1 Subunit Delta	Protein Coding	1
206	ATP5F1E	ATP Synthase F1 Subunit Epsilon	Protein Coding	1
207	ATP5PB	ATP Synthase Peripheral Stalk-Membrane Subunit B	Protein Coding	1
208	ATP5MC1	ATP Synthase Membrane Subunit C Locus 1	Protein Coding	1
209	PDHA1	Pyruvate Dehydrogenase E1 Subunit Alpha 1	Protein Coding	1
210	PDHA2	Pyruvate Dehydrogenase E1 Subunit Alpha 2	Protein Coding	1
211	PDHB	Pyruvate Dehydrogenase E1 Subunit Beta	Protein Coding	1
212	PDK1	Pyruvate Dehydrogenase Kinase 1	Protein Coding	1
213	PDK2	Pyruvate Dehydrogenase Kinase 2	Protein Coding	1
214	PDK3	Pyruvate Dehydrogenase Kinase 3	Protein Coding	1
215	PDK4	Pyruvate Dehydrogenase Kinase 4	Protein Coding	1
216	MPC1	Mitochondrial Pyruvate Carrier 1	Protein Coding	1
217	ATP5MC2	ATP Synthase Membrane Subunit C Locus 2	Protein Coding	1
218	ATP5MC3	ATP Synthase Membrane Subunit C Locus 3	Protein Coding	1
219	ATP5ME	ATP Synthase Membrane Subunit E	Protein Coding	1
220	ATP5PF	ATP Synthase Peripheral Stalk Subunit F6	Protein Coding	1
221	PKLR	Pyruvate Kinase L/R	Protein Coding	1
222	PKM	Pyruvate Kinase M1/2	Protein Coding	1
223	ATP5PO	ATP Synthase Peripheral Stalk Subunit OSCP	Protein Coding	1
224	PDP1	Pyruvate Dehydrogenase Phosphatase Catalytic Subunit 1	Protein Coding	1
225	SDHAF2	Succinate Dehydrogenase Complex Assembly Factor 2	Protein Coding	1
226	GID8	GID Complex Subunit 8 Homolog	Protein Coding	1
227	PDPR	Pyruvate Dehydrogenase Phosphatase Regulatory Subunit	Protein Coding	1
228	SDHAF3	Succinate Dehydrogenase Complex Assembly Factor 3	Protein Coding	1
229	PDP2	Pyruvate Dehydrogenase Phosphatase Catalytic Subunit 2	Protein Coding	1
230	RPS27A	Ribosomal Protein S27a	Protein Coding	1
231	SDHAF1	Succinate Dehydrogenase Complex Assembly Factor 1	Protein Coding	1
232	RMND5B	Required For Meiotic Nuclear Division 5 Homolog B	Protein Coding	1
233	RMND5A	Required For Meiotic Nuclear Division 5 Homolog A	Protein Coding	1
234	D2HGDH	D-2-Hydroxyglutarate Dehydrogenase	Protein Coding	1
235	UBA52	Ubiquitin A-52 Residue Ribosomal Protein Fusion Product 1	Protein Coding	1
236	UBB	Ubiquitin B	Protein Coding	1
237	UBC	Ubiquitin C	Protein Coding	1
238	UCP1	Uncoupling Protein 1	Protein Coding	1
239	UCP2	Uncoupling Protein 2	Protein Coding	1
240	UCP3	Uncoupling Protein 3	Protein Coding	1
241	VDAC1	Voltage Dependent Anion Channel 1	Protein Coding	1
242	CSKMT	Citrate Synthase Lysine Methyltransferase	Protein Coding	1
243	GID4	GID Complex Subunit 4 Homolog	Protein Coding	1
244	L2HGDH	L-2-Hydroxyglutarate Dehydrogenase	Protein Coding	1
245	WDR26	WD Repeat Domain 26	Protein Coding	1
246	PDHX	Pyruvate Dehydrogenase Complex Component X	Protein Coding	1
247	ISCA1	Iron-Sulfur Cluster Assembly 1	Protein Coding	1
248	FAHD1	Fumarylacetoacetate Hydrolase Domain Containing 1	Protein Coding	1
249	ATP5MK	ATP Synthase Membrane Subunit K	Protein Coding	1
250	SUCLG2	Succinate-CoA Ligase GDP-Forming Subunit Beta	Protein Coding	1
251	SUCLG1	Succinate-CoA Ligase GDP/ADP-Forming Subunit Alpha	Protein Coding	1
252	SUCLA2	Succinate-CoA Ligase ADP-Forming Subunit Beta	Protein Coding	1
253	SLC25A14	Solute Carrier Family 25 Member 14	Protein Coding	1
254	KGD4	Alpha-Ketoglutarate Dehydrogenase Subunit 4	Protein Coding	1
255	LDHAL6B	Lactate Dehydrogenase A Like 6B	Protein Coding	1
256	SLC25A27	Solute Carrier Family 25 Member 27	Protein Coding	1
257	ATP5MF	ATP Synthase Membrane Subunit F	Protein Coding	1
258	ATP5MJ	ATP Synthase Membrane Subunit J	Protein Coding	1

Disorders associated with Respiratory electron transport SuperPath

according to GeneCards Suite gene sharing

#	Disorder	Type	Genes	Score
1	Mitochondrial complex i deficiency, nuclear type 1	Enrichment	ACAD9, FOXRED1, MT-ND1, MT-ND2, MT-ND3, MT-ND4, NDUFA1, NDUFA11, NDUFA13, NDUFA6, NDUFAF1, NDUFAF2, NDUFAF3, NDUFAF4, NDUFAF5, NDUFAF8, NDUFB10, NDUFB11, NDUFB3, NDUFB9, NDUFS1, NDUFS2, NDUFS3, NDUFS4, NDUFS6, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NUBPL, TIMMDC1, TMEM126B	16.00
2	Leigh syndrome, nuclear	Enrichment	BCS1L, COX15, FOXRED1, MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, MT-ND6, NDUFA13, NDUFAF2, NDUFAF5, NDUFAF6, NDUFS4, NDUFS7, NDUFV1, SDHA, SURF1	16.00
3	Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes	Enrichment	MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, MT-ND6, NDUFS1	16.00
4	Mitochondrial complex iv deficiency, nuclear type 1	Enrichment	COA3, COX4I1, COX6B1, COX8A, MT-CO1, MT-CO2, MT-CO3, NDUFV1, PET100, SCO1, SURF1, TACO1	16.00
5	Mitochondrial complex v deficiency, mitochondrial type 1	Enrichment	MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5	16.00
6	Neuropathy, ataxia, and retinitis pigmentosa	Enrichment	MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5	16.00
7	Striatonigral degeneration, infantile, mitochondrial	Enrichment	MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5	16.00
8	Mitochondrial myopathy, infantile, transient	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND5	16.00
9	Cox deficiency, benign infantile mitochondrial myopathy	Enrichment	COA3, COX14, COX16, COX20, COX4I1, COX5A, COX6A2, COX6B1, COX8A, MT-CO1, MT-CO2, MT-CO3, PET117	16.00
10	Camptodactyly of fingers	Enrichment	MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5	16.00
11	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	16.00
12	Mitochondrial disease	Enrichment	ATP5F1D, FOXRED1, MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, MT-ND6, NDUFA6, NDUFAF8, NDUFC2, NDUFS1, NDUFS4, OXA1L, PDHX, PYURF, SURF1, TMEM126B, TTC19, UQCRC2	16.00
13	Leber hereditary optic neuropathy, modifier of	Enrichment	MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, MT-ND6, NDUFS2	11.56
14	Isolated complex iii deficiency	Enrichment	BCS1L, CYC1, LYRM7, MT-CYB, TTC19, UQCC2, UQCC3, UQCRB, UQCRC2, UQCRFS1, UQCRQ	11.41
15	Leigh disease	Enrichment	ATP5PO, BCS1L, COX15, FOXRED1, MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, MT-ND6, NDUFA13, NDUFAF2, NDUFAF5, NDUFAF6, NDUFS1, NDUFS4, NDUFS7, NDUFS8, NDUFV1, SDHA, SURF1	11.12
16	Hereditary paraganglioma-pheochromocytoma syndromes	Enrichment	DLST, FH, MDH2, SDHA, SDHAF2, SDHB, SDHC, SDHD, SLC25A11	10.25
17	Leber optic atrophy and dystonia	Enrichment	MT-ND1, MT-ND3, MT-ND4, MT-ND5, MT-ND6	9.67
18	Leigh syndrome, mitochondrial	Enrichment	MT-ATP6, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, MT-ND6	9.58
19	Leber plus disease	Enrichment	MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, MT-ND6, NDUFAF5, NDUFS2	9.04
20	Isolated atp synthase deficiency	Enrichment	ATP5F1A, ATP5F1D, ATP5F1E, ATP5MK, MT-ATP6, MT-ATP8	8.91
21	Hypertelorism	Enrichment	MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, RAB5IF	8.54
22	Cox deficiency, infantile mitochondrial myopathy	Enrichment	COA5, COX15, SCO1, SCO2	7.07
23	Mitochondrial complex ii deficiency, nuclear type 1	Enrichment	NDUFS8, SDHA, SDHAF1, SDHD	6.89
24	Cardiomyopathy, infantile histiocytoid	Enrichment	MT-ATP6, MT-ATP8, MT-CYB, NDUFB11	6.89
25	Mitochondrial complex ii deficiency	Enrichment	SDHA, SDHAF1, SDHB, SDHD	6.89
26	Mitochondrial complex iii deficiency, nuclear type 1	Enrichment	BCS1L, LYRM7, NDUFS7	5.82
27	Linear skin defects with multiple congenital anomalies 1	Enrichment	COX7B, HCCS, NDUFB11	5.82
28	Carney triad	Enrichment	SDHA, SDHB, SDHC	5.82
29	Paraganglioma	Enrichment	SDHA, SDHB, SDHD	5.82
30	Lactic acidosis	Enrichment	ATP5F1A, DLD, NDUFA13, NDUFS4, UQCRFS1	5.77
31	Pheochromocytoma/paraganglioma syndrome 1	Enrichment	SDHA, SDHB, SDHD	5.22
32	Multiple acyl-coa dehydrogenase deficiency	Enrichment	ETFA, ETFB, ETFDH	5.22
33	Mitochondrial complex v deficiency, nuclear type 5	Enrichment	ATP5F1D, MT-ND5, NDUFV1	5.17
34	Paraganglioma and gastric stromal sarcoma	Enrichment	SDHB, SDHC, SDHD	4.83
35	Multiple acyl-coa dehydrogenase deficiency, severe neonatal type	Enrichment	ETFA, ETFB, ETFDH	4.83
36	Multiple acyl-coa dehydrogenase deficiency, mild type	Enrichment	ETFA, ETFB, ETFDH	4.83
37	Gastrointestinal stromal tumor	Enrichment	SDHA, SDHB, SDHC	4.29
38	Developmental and epileptic encephalopathy 1	Enrichment	MDH2, SLC25A12	3.93
39	Cowden syndrome	Enrichment	SDHB, SDHC, SDHD	3.92
40	Mitochondrial complex iv deficiency, nuclear type 6	Enrichment	COX15, SURF1	3.88
41	Alzheimer disease mitochondrial	Enrichment	MT-ND1, MT-ND2	3.88
42	Optic atrophy plus syndrome	Enrichment	ACO2, ISCA2, MT-ND1, MT-ND4, MT-ND6, TMEM126A	3.76
43	Familial colorectal cancer	Enrichment	MT-CO1, MT-CO2, MT-CYB	3.76
44	Rare mitochondrial non-syndromic sensorineural deafness	Enrichment	MT-CO1, MT-ND1, MT-ND4	3.76
45	Retinitis pigmentosa	Enrichment	ATP5ME, IDH3A, IDH3B, MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5	3.67
46	Developmental and epileptic encephalopathy	Enrichment	GOT2, SLC25A22	3.61
47	L-2-hydroxyglutaric aciduria	Enrichment	DMAC2L, L2HGDH	3.44
48	Cardiomyopathy, infantile hypertrophic	Enrichment	MT-ATP6, MT-ATP8	3.44
49	Multiple mitochondrial dysfunctions syndrome	Enrichment	ISCA1, ISCA2	3.44
50	D-2-hydroxyglutaric aciduria	Enrichment	D2HGDH, IDH2	3.44
51	Periodic paralysis with later-onset distal motor neuropathy	Enrichment	MT-ATP6, MT-ATP8	3.44
52	West syndrome	Enrichment	MDH2, SLC25A12	3.44
53	Pheochromocytoma/paraganglioma syndrome 4	Enrichment	SDHB, SDHD	3.40
54	Mitochondrial complex iv deficiency, nuclear type 2	Enrichment	SCO2, SDHB	3.40
55	Mitochondrial complex iii deficiency, nuclear type 2	Enrichment	SDHD, TTC19	3.40
56	Combined oxidative phosphorylation deficiency 19	Enrichment	LYRM4, NFS1	3.40
57	Pheochromocytoma	Enrichment	SDHA, SDHB, SDHD	3.30
58	Developmental and epileptic encephalopathy 39 with leukodystrophy	Enrichment	SLC25A12	3.18
59	Developmental and epileptic encephalopathy 3	Enrichment	SLC25A22	3.18
60	Aspartate aminotransferase, serum level of, quantitative trait locus 1	Enrichment	GOT1	3.18
61	Developmental and epileptic encephalopathy 51	Enrichment	MDH2	3.18
62	Pheochromocytoma/paraganglioma syndrome 6	Enrichment	SLC25A11	3.18
63	Developmental and epileptic encephalopathy 39	Enrichment	SLC25A12	3.18
64	Citrullinemia type ii	Enrichment	SLC25A13	3.18
65	Optic nerve disease	Enrichment	MT-ATP6, MT-ATP8, MT-ND1	3.13
66	Hereditary recurrent myoglobinuria	Enrichment	MT-CO1, MT-CO3	3.10
67	Citrullinemia, classic	Enrichment	SLC25A13	2.88
68	Citrin deficiency, neonatal or infantile onset	Enrichment	SLC25A13	2.88
69	Developmental and epileptic encephalopathy 82	Enrichment	GOT2	2.88
70	Developmental and epileptic encephalopathy 88	Enrichment	MDH1	2.88
71	Citrullinemia	Enrichment	SLC25A13	2.88
72	Deafness, nonsyndromic sensorineural, mitochondrial	Enrichment	MT-CO1, MT-ND1	2.71
73	Parkinson disease 6, autosomal recessive early-onset	Enrichment	MT-ND5, MT-ND6	2.71
74	Sporadic pheochromocytoma/secreting paraganglioma	Enrichment	SDHB, SDHD	2.71
75	Citrin deficiency, adolescent or adult onset	Enrichment	SLC25A13	2.70
76	Pyruvate dehydrogenase e1-alpha deficiency	Enrichment	PDHA1, PDHX	2.68
77	Mitochondrial complex v deficiency, nuclear type 3	Enrichment	ATP5F1E, UQCRC2	2.68
78	Tetralogy of fallot	Enrichment	MT-CO1, MT-CO2, MT-CO3	2.49
79	Early myoclonic encephalopathy	Enrichment	SLC25A22	2.40
80	Developmental and epileptic encephalopathy 14	Enrichment	SLC25A22	2.22
81	Linear skin defects with multiple congenital anomalies 2	Enrichment	COX7B	1.94
82	Mitochondrial complex i deficiency, nuclear type 30	Enrichment	NDUFB11	1.94
83	Mitochondrial complex i deficiency, mitochondrial type 1	Enrichment	MT-ND3	1.94
84	Myoglobinuria, recurrent	Enrichment	MT-CO1	1.94
85	Mitochondrial complex iii deficiency, nuclear type 3	Enrichment	UQCRB	1.94
86	Mitochondrial complex iii deficiency, nuclear type 6	Enrichment	CYC1	1.94
87	Mitochondrial complex iii deficiency, nuclear type 7	Enrichment	UQCC2	1.94
88	Pheochromocytoma/paraganglioma syndrome 3	Enrichment	SDHC	1.94
89	Myopathy with lactic acidosis, hereditary	Enrichment	ISCU	1.94
90	Even-plus syndrome	Enrichment	HSPA9	1.94
91	Pheochromocytoma/paraganglioma syndrome 5	Enrichment	SDHA	1.94
92	Anemia, sideroblastic, 4	Enrichment	HSPA9	1.94
93	Mitochondrial complex i deficiency, nuclear type 37	Enrichment	NDUFA8	1.94
94	Bjornstad syndrome	Enrichment	BCS1L	1.94
95	Mitochondrial complex iv deficiency, nuclear type 8	Enrichment	TACO1	1.94
96	Mitochondrial complex iv deficiency, nuclear type 18	Enrichment	COX6A2	1.94
97	Combined oxidative phosphorylation deficiency 52	Enrichment	NFS1	1.94
98	Mitochondrial complex i deficiency, nuclear type 9	Enrichment	NDUFS6	1.94
99	Mitochondrial complex i deficiency, nuclear type 23	Enrichment	NDUFA12	1.94
100	Mitochondrial complex i deficiency, nuclear type 25	Enrichment	NDUFB3	1.94
101	Mitochondrial complex i deficiency, nuclear type 31	Enrichment	TIMMDC1	1.94
102	Mitochondrial complex i deficiency, nuclear type 19	Enrichment	FOXRED1	1.94
103	Mitochondrial complex i deficiency, nuclear type 22	Enrichment	NDUFA10	1.94
104	Neurodegeneration, childhood-onset, with multisystem involvement due to mitochondrial dysfunction	Enrichment	LETM1	1.94
105	Fanconi renotubular syndrome 5	Enrichment	NDUFAF6	1.94
106	Mitochondrial complex iv deficiency, nuclear type 10	Enrichment	COX14	1.94
107	Mitochondrial complex iv deficiency, nuclear type 14	Enrichment	COA3	1.94
108	Mitochondrial complex i deficiency, nuclear type 8	Enrichment	NDUFS3	1.94
109	Mitochondrial complex i deficiency, nuclear type 11	Enrichment	NDUFAF1	1.94
110	Mitochondrial complex iv deficiency, nuclear type 23	Enrichment	COX11	1.94
111	Mitochondrial complex i deficiency, nuclear type 17	Enrichment	NDUFAF6	1.94
112	Mitochondrial complex i deficiency, nuclear type 26	Enrichment	NDUFA9	1.94
113	Mitochondrial complex i deficiency, nuclear type 29	Enrichment	TMEM126B	1.94
114	Linear skin defects with multiple congenital anomalies 3	Enrichment	NDUFB11	1.94
115	Oncocytoma	Enrichment	MT-ND6	1.94
116	Gracile syndrome	Enrichment	BCS1L	1.94
117	Mitochondrial complex iii deficiency, nuclear type 8	Enrichment	LYRM7	1.94
118	Cardiomyopathy, dilated, 1gg	Enrichment	SDHA	1.94
119	Optic atrophy 7 with or without auditory neuropathy	Enrichment	TMEM126A	1.94
120	Mitochondrial complex iii deficiency, nuclear type 10	Enrichment	UQCRFS1	1.94
121	Combined oxidative phosphorylation deficiency 18	Enrichment	SFXN4	1.94
122	Exocrine pancreatic insufficiency, dyserythropoietic anemia, and calvarial hyperostosis	Enrichment	COX4I2	1.94
123	Mitochondrial complex i deficiency, nuclear type 24	Enrichment	NDUFB9	1.94
124	Mitochondrial complex i deficiency, nuclear type 33	Enrichment	NDUFA6	1.94
125	Neurodegeneration with ataxia and late-onset optic atrophy	Enrichment	SDHA	1.94
126	Mitochondrial complex iv deficiency, nuclear type 9	Enrichment	COA5	1.94
127	Mitochondrial complex iv deficiency, nuclear type 7	Enrichment	COX6B1	1.94
128	Mitochondrial complex iv deficiency, nuclear type 20	Enrichment	COX5A	1.94
129	Mitochondrial complex iii deficiency, nuclear type 11	Enrichment	UQCRH	1.94
130	Mitochondrial complex i deficiency, nuclear type 6	Enrichment	NDUFS2	1.94
131	Mitochondrial complex i deficiency, nuclear type 21	Enrichment	NUBPL	1.94
132	Mitochondrial complex i deficiency, nuclear type 32	Enrichment	NDUFB8	1.94
133	Mitochondrial complex iv deficiency, nuclear type 16	Enrichment	COX4I1	1.94
134	Mitochondrial complex iv deficiency, nuclear type 21	Enrichment	COXFA4	1.94
135	Mitochondrial complex iv deficiency, nuclear type 15	Enrichment	COX8A	1.94
136	Autosomal recessive optic atrophy, opa7 type	Enrichment	TMEM126A	1.94
137	Anemia, sideroblastic, 5	Enrichment	HSCB	1.94
138	Charcot-marie-tooth disease, recessive intermediate d	Enrichment	COX6A1	1.94
139	Mitochondrial complex i deficiency, nuclear type 39	Enrichment	NDUFB7	1.94
140	Leber-like hereditary optic neuropathy, autosomal recessive 2	Enrichment	NDUFS2	1.94
141	Autosomal recessive axonal charcot-marie-tooth disease due to copper metabolism defect	Enrichment	SCO2	1.94
142	Pancreatic insufficiency-anemia-hyperostosis syndrome	Enrichment	COX4I2	1.94
143	Progressive cavitating leukoencephalopathy	Enrichment	NDUFV2	1.94
144	Carcinoid syndrome	Enrichment	SDHD	1.94
145	Autosomal dominant spastic ataxia	Enrichment	MT-CO3	1.94
146	Renal tubulopathy-encephalopathy-liver failure syndrome	Enrichment	BCS1L	1.94
147	Mitochondrial disorder due to a defect in assembly or maturation of the respiratory chain complexes	Enrichment	NDUFB7	1.94
148	Early infantile developmental and epileptic encephalopathy	Enrichment	SLC25A22	1.93
149	Hypertrophic cardiomyopathy	Enrichment	ETFDH, NDUFA13, UQCRC1	1.87
150	Rhabdomyosarcoma	Enrichment	SDHA, SDHC	1.84
151	Adenosine triphosphate, elevated, of erythrocytes	Enrichment	PKLR	1.72
152	Hereditary leiomyomatosis and renal cell cancer	Enrichment	FH	1.72
153	Mitochondrial dna depletion syndrome 9	Enrichment	SUCLG1	1.72
154	Anemia, congenital, nonspherocytic hemolytic, 2	Enrichment	PKLR	1.72
155	Oxoglutarate dehydrogenase deficiency	Enrichment	OGDH	1.72
156	Ataxia and polyneuropathy, adult-onset	Enrichment	MT-ATP6	1.72
157	Retinitis pigmentosa 99	Enrichment	IDH3G	1.72
158	Pheochromocytoma/paraganglioma syndrome 2	Enrichment	SDHAF2	1.72
159	Charcot-marie-tooth disease, x-linked dominant, 6	Enrichment	PDK3	1.72
160	Myopathy, lactic acidosis, and sideroblastic anemia 3	Enrichment	MT-ATP6	1.72
161	Fumarase deficiency	Enrichment	FH	1.72
162	Hydroxyacyl glutathione hydrolase deficiency	Enrichment	HAGH	1.72
163	Multiple mitochondrial dysfunctions syndrome 4	Enrichment	ISCA2	1.72
164	Dystonia, early-onset, and/or spastic paraplegia	Enrichment	ATP5MC3	1.72
165	Retinitis pigmentosa 90	Enrichment	IDH3A	1.72
166	Mitochondrial complex v deficiency, nuclear type 6	Enrichment	ATP5MK	1.72
167	Body mass index quantitative trait locus 4	Enrichment	UCP2	1.72
168	Pyruvate dehydrogenase phosphatase deficiency	Enrichment	PDP1	1.72
169	Pheochromocytoma/paraganglioma syndrome 7	Enrichment	DLST	1.72
170	Mitochondrial complex v deficiency, nuclear type 4b	Enrichment	ATP5F1A	1.72
171	Mitochondrial dna depletion syndrome 5	Enrichment	SUCLA2	1.72
172	Retinitis pigmentosa 46	Enrichment	IDH3B	1.72
173	Leiomyoma cutis	Enrichment	FH	1.72
174	Gemistocytic astrocytoma	Enrichment	IDH2	1.72
175	Protoplasmic astrocytoma	Enrichment	IDH2	1.72
176	Lactate dehydrogenase b deficiency	Enrichment	LDHB	1.72
177	Glucocorticoid deficiency 4 with or without mineralocorticoid deficiency	Enrichment	NNT	1.72
178	D-2-hydroxyglutaric aciduria 2	Enrichment	IDH2	1.72
179	Combined oxidative phosphorylation deficiency 22	Enrichment	ATP5F1A	1.72
180	Spermatogenic failure 70	Enrichment	PDHA2	1.72
181	Mitochondrial pyruvate carrier deficiency	Enrichment	MPC1	1.72
182	Fumarate hydratase deficiency	Enrichment	FH	1.72
183	Mixed oligodendroglioma-astrocytoma	Enrichment	IDH2	1.72
184	Anaplastic oligoastrocytoma	Enrichment	IDH2	1.72
185	Maleylacetoacetate isomerase deficiency	Enrichment	GSTZ1	1.72
186	Amyotrophic lateral sclerosis 24	Enrichment	NEK1	1.72
187	Mitochondrial complex v deficiency, nuclear type 4a	Enrichment	ATP5F1A	1.72
188	Hypermetabolism due to uncoupled mitochondrial oxidative phosphorylation 2	Enrichment	ATP5F1B	1.72
189	Fibrillary astrocytoma	Enrichment	IDH2	1.72
190	Acute myocardial infarction	Enrichment	IDH2	1.72
191	Suclg1-related mitochondrial dna depletion syndrome, encephalomyopathic form with methylmalonic aciduria	Enrichment	SUCLG1	1.72
192	Hyperinsulinism due to ucp2 deficiency	Enrichment	UCP2	1.72
193	Maternally-inherited spastic paraplegia	Enrichment	MT-ATP6	1.72
194	Deafness, sensorineural, autosomal-mitochondrial type	Enrichment	MT-ND1	1.64
195	Polymyoclonus, infantile	Enrichment	SDHA	1.64
196	Thyroid carcinoma, hurthle cell	Enrichment	NDUFA13	1.64
197	Leukoencephalopathy, cystic, without megalencephaly	Enrichment	NDUFA2	1.64
198	Glutaric aciduria iii	Enrichment	ETFA	1.64
199	Hyperphosphatasia with impaired intellectual development syndrome 6	Enrichment	PYURF	1.64
200	Mitochondrial complex iii deficiency, nuclear type 9	Enrichment	UQCC3	1.64
201	Mitochondrial complex iv deficiency, nuclear type 19	Enrichment	PET117	1.64
202	Mitochondrial complex i deficiency, nuclear type 10	Enrichment	NDUFAF2	1.64
203	Mitochondrial complex i deficiency, nuclear type 15	Enrichment	NDUFAF4	1.64
204	Mitochondrial complex i deficiency, nuclear type 14	Enrichment	NDUFA11	1.64
205	Myopia 6	Enrichment	SCO2	1.64
206	Mitochondrial complex i deficiency, nuclear type 12	Enrichment	NDUFA1	1.64
207	Mitochondrial complex i deficiency, nuclear type 36	Enrichment	NDUFC2	1.64
208	Charcot-marie-tooth disease type 4k	Enrichment	SURF1	1.64
209	Mitochondrial complex iv deficiency, nuclear type 11	Enrichment	COX20	1.64
210	Mitochondrial complex iv deficiency, nuclear type 12	Enrichment	PET100	1.64
211	Charcot-marie-tooth disease, demyelinating, type 4k	Enrichment	SURF1	1.64
212	Mitochondrial complex iv deficiency, nuclear type 22	Enrichment	COX16	1.64
213	Autosomal recessive sideroblastic anemia	Enrichment	HSPA9	1.64
214	Mitochondrial complex iv deficiency, nuclear type 4	Enrichment	SCO1	1.64
215	Nuclear type mitochondrial complex i deficiency	Enrichment	NDUFV1	1.64
216	Mitochondrial complex i deficiency, nuclear type 13	Enrichment	NDUFA2	1.64
217	Mitochondrial complex i deficiency, nuclear type 16	Enrichment	NDUFAF5	1.64
218	Mitochondrial complex i deficiency, nuclear type 7	Enrichment	NDUFV2	1.64
219	Parkinsonism with polyneuropathy	Enrichment	UQCRC1	1.64
220	Paroxysmal dyskinesia	Enrichment	PNKD	1.64
221	Aortic valve disease 1	Enrichment	MT-ATP6, MT-ATP8	1.61
222	Keratoderma, palmoplantar, with deafness	Enrichment	MT-CO1	1.46
223	Mitochondrial myopathy, episodic, with or without optic atrophy and reversible leukoencephalopathy	Enrichment	MT-ND3	1.46
224	Friedreich ataxia	Enrichment	FXN	1.46
225	Mitochondrial complex i deficiency, nuclear type 20	Enrichment	ACAD9	1.46
226	Uv-sensitive syndrome 2	Enrichment	NDUFAF2	1.46
227	Craniofacial dysmorphism, skeletal anomalies, and impaired intellectual development syndrome 2	Enrichment	RAB5IF	1.46
228	Mitochondrial complex i deficiency, nuclear type 34	Enrichment	NDUFAF8	1.46
229	Mitochondrial complex v deficiency, nuclear type 1	Enrichment	NDUFS1	1.46
230	Mitochondrial complex i deficiency, nuclear type 35	Enrichment	NDUFB10	1.46
231	Brain cancer	Enrichment	SDHA	1.46
232	Mitochondrial complex i deficiency, nuclear type 28	Enrichment	NDUFA13	1.46
233	Myelodysplastic syndrome with ring sideroblasts	Enrichment	MT-CO1	1.46
234	Renal cell carcinoma	Enrichment	SDHB	1.46
235	Charcot-marie-tooth disease, demyelinating, type 1a	Enrichment	MT-ATP6	1.42
236	Leiomyoma, uterine	Enrichment	FH	1.42
237	Myeloproliferative disorder, chronic, with eosinophilia	Enrichment	MAEA	1.42
238	Fanconi-bickel syndrome	Enrichment	LDHA	1.42
239	Alpha-methylacetoacetic aciduria	Enrichment	ACAT1	1.42
240	Orofaciodigital syndrome ii	Enrichment	NEK1	1.42
241	Dihydrolipoamide dehydrogenase deficiency	Enrichment	DLD	1.42
242	Porphyria, acute intermittent	Enrichment	ACO2	1.42
243	Methemoglobinemia due to deficiency of methemoglobin reductase	Enrichment	DLD	1.42
244	Pyruvate dehydrogenase e2 deficiency	Enrichment	DLAT	1.42
245	Dyskeratosis congenita, autosomal recessive 2	Enrichment	RMND5B	1.42
246	Pyruvate dehydrogenase e1-beta deficiency	Enrichment	PDHB	1.42
247	Pyruvate dehydrogenase e3-binding protein deficiency	Enrichment	PDHX	1.42
248	D-2-hydroxyglutaric aciduria 1	Enrichment	D2HGDH	1.42
249	Multiple mitochondrial dysfunctions syndrome 5	Enrichment	ISCA1	1.42
250	Charcot-marie-tooth disease type 1a	Enrichment	MT-ATP6	1.42
251	Oligodendroglioma	Enrichment	IDH2	1.42
252	Skraban-deardorff syndrome	Enrichment	WDR26	1.42
253	Acute porphyria	Enrichment	ACO2	1.42
254	Spermatogenic failure 22	Enrichment	FAHD1	1.42
255	Anaplastic oligodendroglioma	Enrichment	IDH2	1.42
256	Spinocerebellar ataxia 45	Enrichment	FH	1.42
257	Premature ovarian failure 23	Enrichment	FAHD1	1.42
258	Submucosal cleft palate	Enrichment	UBB	1.42
259	Cleft hard palate	Enrichment	UBB	1.42
260	Inherited cancer-predisposing syndrome	Enrichment	FH, SDHA, SDHAF2, SDHB, SDHC, SDHD	1.39
261	Sudden infant death syndrome	Enrichment	MT-ND1, PDHA1	1.39
262	Paroxysmal nonkinesigenic dyskinesia 1	Enrichment	PNKD	1.34
263	Craniofacial dysmorphism, skeletal anomalies, and impaired intellectual development syndrome 1	Enrichment	RAB5IF	1.34
264	Propionic acidemia	Enrichment	UQCRFS1	1.34
265	Mitochondrial complex i deficiency, nuclear type 18	Enrichment	NDUFAF3	1.34
266	Pilocytic astrocytoma	Enrichment	SDHA	1.34
267	Familial paroxysmal nonkinesigenic dyskinesia	Enrichment	PNKD	1.34
268	Primary fanconi renotubular syndrome	Enrichment	NDUFAF6	1.34
269	Azoospermia	Enrichment	FAHD1, PDHA2	1.26
270	Mitochondrial complex iv deficiency, nuclear type 5	Enrichment	ATP5F1A	1.25
271	Pyruvate carboxylase deficiency	Enrichment	PC	1.25
272	Uvula, bifid	Enrichment	UBB	1.25
273	Cleft soft palate	Enrichment	UBB	1.25
274	Infantile cerebellar-retinal degeneration	Enrichment	ACO2	1.25
275	Optic atrophy 9	Enrichment	ACO2	1.25
276	Retinitis pigmentosa 40	Enrichment	ATP5ME	1.25
277	Mitochondrial complex ii deficiency, nuclear type 2	Enrichment	SDHAF1	1.25
278	Mitochondrial complex v deficiency, nuclear type 7	Enrichment	ATP5PO	1.25
279	Renu syndrome	Enrichment	SIRT4	1.25
280	Anaplastic astrocytoma	Enrichment	IDH2	1.25
281	Gonadal dysgenesis	Enrichment	MT-ATP6	1.25
282	Cockayne syndrome a	Enrichment	NDUFAF2	1.25
283	Von hippel-lindau syndrome	Enrichment	SDHB	1.25
284	Mitochondrial complex iii deficiency, nuclear type 4	Enrichment	UQCRQ	1.25
285	Rare isolated myopia	Enrichment	SCO2	1.25
286	Mitochondrial oxidative phosphorylation disorder	Enrichment	NUBPL	1.25
287	Dystonia	Enrichment	MT-ND1, MT-ND6	1.21
288	Wolf-hirschhorn syndrome	Enrichment	LETM1	1.17
289	Mitochondrial dna depletion syndrome 1	Enrichment	SCO2	1.17
290	Congenital anomalies of kidney and urinary tract 1	Enrichment	TRAP1	1.17
291	Body mass index quantitative trait locus 11	Enrichment	MT-CYB, UCP2, UCP3	1.16
292	Spermatogenic failure 1	Enrichment	PDHA2	1.13
293	Leptin deficiency or dysfunction	Enrichment	UCP3	1.13
294	Spastic quadriplegic cerebral palsy	Enrichment	ISCA2	1.13
295	Autosomal recessive isolated optic atrophy	Enrichment	ACO2	1.13
296	Familial infantile bilateral striatal necrosis	Enrichment	MT-ATP6	1.13
297	Epilepsy	Enrichment	MT-CO3, MT-CYB	1.11
298	Parkinsonism	Enrichment	MT-ND6	1.11
299	Cockayne syndrome	Enrichment	NDUFAF2	1.11
300	Paroxysmal dystonia	Enrichment	PNKD	1.11
301	Mitochondrial dna depletion syndrome 4b	Enrichment	SCO2	1.05
302	Enchondromatosis, multiple, ollier type	Enrichment	IDH2	1.04
303	Albinism, oculocutaneous, type ii	Enrichment	ATP5ME	1.04
304	Kearns-sayre syndrome	Enrichment	MT-ATP8	1.04
305	Familial glucocorticoid deficiency	Enrichment	NNT	1.04
306	Hereditary breast carcinoma	Enrichment	MT-CYB, SDHA	1.04
307	Mitochondrial neurogastrointestinal encephalomyopathy	Enrichment	SCO2	1.00
308	Short rib-polydactyly syndrome	Enrichment	NEK1	0.96
309	Mitochondrial encephalomyopathy	Enrichment	MT-CYB	0.96
310	Migraine with or without aura 1	Enrichment	MT-ND1	0.92
311	Movement disease	Enrichment	BCS1L	0.92
312	Multiple enchondromatosis, maffucci type	Enrichment	IDH2	0.90
313	Motor neuron disease	Enrichment	NEK1	0.90
314	Hemolytic anemia	Enrichment	PKLR	0.90
315	Myoclonic epilepsy associated with ragged-red fibers	Enrichment	MT-ND5	0.88
316	Restrictive cardiomyopathy	Enrichment	MT-ND1	0.88
317	Alzheimer's disease	Enrichment	MT-ND1	0.85
318	Glioma susceptibility 1	Enrichment	IDH2	0.85
319	Short-rib thoracic dysplasia 12	Enrichment	NEK1	0.85
320	Hydrocephalus, congenital, 1	Enrichment	MT-ND1	0.77
321	Alzheimer disease, familial, 1	Enrichment	MT-ND1	0.75
322	Parkinson's disease	Enrichment	MT-ND1	0.70
323	Short-rib thoracic dysplasia 6 with or without polydactyly	Enrichment	NEK1	0.69
324	Short-rib thoracic dysplasia 3 with or without polydactyly	Enrichment	NEK1	0.69
325	Breast cancer	Enrichment	MT-CYB, SDHA	0.67
326	Attention deficit-hyperactivity disorder	Enrichment	MT-ND1	0.65
327	Isolated macular dystrophy	Enrichment	ACO2	0.63
328	Parkinson disease, late-onset	Enrichment	MT-ND1	0.61
329	Scoliosis	Enrichment	RAB5IF	0.61
330	Auditory neuropathy	Enrichment	MT-ND6	0.58
331	Colorectal cancer	Enrichment	MT-CO1, MT-ND1	0.57
332	Differentiated thyroid carcinoma	Enrichment	NDUFA13	0.54
333	Ovarian cancer	Enrichment	FH, MT-CYB, SDHAF2	0.52
334	Peripheral nervous system disease	Enrichment	COX6A1	0.51
335	Neuropathy	Enrichment	COX6A1	0.51
336	Cakut	Enrichment	TRAP1	0.49
337	Hepatocellular carcinoma	Enrichment	FH	0.46
338	Connective tissue disease	Enrichment	NEK1	0.34
339	Familial isolated dilated cardiomyopathy	Enrichment	SDHA	0.34
340	Leukemia, acute myeloid	Enrichment	IDH2	0.27
341	Charcot-marie-tooth disease	Enrichment	MT-ATP6	0.26
342	Rare genetic deafness	Enrichment	MT-ND1	0.22
343	Dilated cardiomyopathy	Enrichment	SCO2	0.22
344	Hereditary retinal dystrophy	Enrichment	ACO2, ATP5ME, IDH3A, MT-ND4, MT-ND6	0.19
345	Fundus dystrophy	Enrichment	ACO2, ATP5ME, IDH3A, MT-ND4, MT-ND6	0.19
346	Male infertility with azoospermia or oligozoospermia due to single gene mutation	Enrichment	PDHA2	0.18
347	Frontotemporal dementia and/or amyotrophic lateral sclerosis 7	Enrichment	NEK1	0.17
348	Microcephaly	Enrichment	ATP5PO, BCS1L	0.17

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

Respiratory electron transport

Pathway Network for Respiratory electron transport

Pathway network for the Respiratory electron transport SuperPath

Member Pathways for Respiratory electron transport

Pathways in the Respiratory electron transport SuperPath

Gene overlap in member pathways for Respiratory electron transport SuperPath

Associated Genes for Respiratory electron transport

Associated Disorders for Respiratory electron transport

Disorders associated with Respiratory electron transport SuperPath

STRING Interaction Network for Respiratory electron transport

Sources for Respiratory electron transport