Complex I biogenesis

No Pathway Network information available for Complex I biogenesis

Pathways in the Complex I biogenesis SuperPath

#	Name	Source	Genes
1	Complex I biogenesis	Reactome	ACAD9 COA1 DMAC1 DMAC2 FOXRED1 HSCB HSPA9 LYRM2 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 NUBPL OXA1L PYURF SFXN4 TIMMDC1 TMEM126A TMEM126B TMEM186 (see all 67) (see less)
2	Mitochondrial complex I assembly model OXPHOS system	WikiPathways	ACAD9 COA1 DMAC1 DMAC2 ECSIT FOXRED1 MT-ND1 MT-ND2 MT-ND4 MT-ND4L MT-ND5 MT-ND6 NDUFA1 NDUFA10 NDUFA12 NDUFA13 NDUFA2 NDUFA3 NDUFA5 NDUFA6 NDUFA7 NDUFA8 NDUFAB1 NDUFAF1 NDUFAF2 NDUFAF3 NDUFAF4 NDUFAF6 NDUFAF7 NDUFB1 NDUFB10 NDUFB11 NDUFB2 NDUFB3 NDUFB4 NDUFB5 NDUFB6 NDUFB7 NDUFB8 NDUFB9 NDUFC1 NDUFC2 NDUFS1 NDUFS2 NDUFS3 NDUFS4 NDUFS5 NDUFS6 NDUFV1 NDUFV2 NDUFV3 NUBPL TIMMDC1 TMEM126B TMEM186 TMEM70 (see all 56) (see less)

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

#	Symbol	Description	Category	# Related Pathways
1	NDUFAF6	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 6	Protein Coding	2
2	TMEM186	Transmembrane Protein 186	Protein Coding	2
3	NDUFAF3	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 3	Protein Coding	2
4	ACAD9	Acyl-CoA Dehydrogenase Family Member 9	Protein Coding	2
5	NDUFAF4	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 4	Protein Coding	2
6	MT-ND1	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 1	Protein Coding	2
7	MT-ND2	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 2	Protein Coding	2
8	MT-ND4	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 4	Protein Coding	2
9	MT-ND5	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 5	Protein Coding	2
10	MT-ND6	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 6	Protein Coding	2
11	NDUFA1	NADH:Ubiquinone Oxidoreductase Subunit A1	Protein Coding	2
12	NDUFA2	NADH:Ubiquinone Oxidoreductase Subunit A2	Protein Coding	2
13	NDUFA3	NADH:Ubiquinone Oxidoreductase Subunit A3	Protein Coding	2
14	NDUFA5	NADH:Ubiquinone Oxidoreductase Subunit A5	Protein Coding	2
15	NDUFA6	NADH:Ubiquinone Oxidoreductase Subunit A6	Protein Coding	2
16	NDUFA7	NADH:Ubiquinone Oxidoreductase Subunit A7	Protein Coding	2
17	NDUFA8	NADH:Ubiquinone Oxidoreductase Subunit A8	Protein Coding	2
18	NDUFA10	NADH:Ubiquinone Oxidoreductase Subunit A10	Protein Coding	2
19	NDUFAB1	NADH:Ubiquinone Oxidoreductase Subunit AB1	Protein Coding	2
20	NDUFB1	NADH:Ubiquinone Oxidoreductase Subunit B1	Protein Coding	2
21	NDUFB2	NADH:Ubiquinone Oxidoreductase Subunit B2	Protein Coding	2
22	NDUFB3	NADH:Ubiquinone Oxidoreductase Subunit B3	Protein Coding	2
23	NDUFB4	NADH:Ubiquinone Oxidoreductase Subunit B4	Protein Coding	2
24	NDUFB5	NADH:Ubiquinone Oxidoreductase Subunit B5	Protein Coding	2
25	NDUFB6	NADH:Ubiquinone Oxidoreductase Subunit B6	Protein Coding	2
26	NDUFB7	NADH:Ubiquinone Oxidoreductase Subunit B7	Protein Coding	2
27	NDUFB8	NADH:Ubiquinone Oxidoreductase Subunit B8	Protein Coding	2
28	NDUFB9	NADH:Ubiquinone Oxidoreductase Subunit B9	Protein Coding	2
29	NDUFB10	NADH:Ubiquinone Oxidoreductase Subunit B10	Protein Coding	2
30	NDUFC1	NADH:Ubiquinone Oxidoreductase Subunit C1	Protein Coding	2
31	NDUFC2	NADH:Ubiquinone Oxidoreductase Subunit C2	Protein Coding	2
32	NDUFS1	NADH:Ubiquinone Oxidoreductase Core Subunit S1	Protein Coding	2
33	NDUFS2	NADH:Ubiquinone Oxidoreductase Core Subunit S2	Protein Coding	2
34	NDUFS3	NADH:Ubiquinone Oxidoreductase Core Subunit S3	Protein Coding	2
35	NDUFV1	NADH:Ubiquinone Oxidoreductase Core Subunit V1	Protein Coding	2
36	NDUFS4	NADH:Ubiquinone Oxidoreductase Subunit S4	Protein Coding	2
37	NDUFS5	NADH:Ubiquinone Oxidoreductase Subunit S5	Protein Coding	2
38	NDUFS6	NADH:Ubiquinone Oxidoreductase Subunit S6	Protein Coding	2
39	NDUFV2	NADH:Ubiquinone Oxidoreductase Core Subunit V2	Protein Coding	2
40	NDUFV3	NADH:Ubiquinone Oxidoreductase Subunit V3	Protein Coding	2
41	NDUFA13	NADH:Ubiquinone Oxidoreductase Subunit A13	Protein Coding	2
42	NDUFAF1	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 1	Protein Coding	2
43	TIMMDC1	Translocase Of Inner Mitochondrial Membrane Domain Containing 1	Protein Coding	2
44	NDUFB11	NADH:Ubiquinone Oxidoreductase Subunit B11	Protein Coding	2
45	DMAC2	Distal Membrane Arm Assembly Component 2	Protein Coding	2
46	NDUFAF7	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 7	Protein Coding	2
47	FOXRED1	FAD Dependent Oxidoreductase Domain Containing 1	Protein Coding	2
48	COA1	Cytochrome C Oxidase Assembly Factor 1	Protein Coding	2
49	TMEM126B	Transmembrane Protein 126B	Protein Coding	2
50	NDUFA12	NADH:Ubiquinone Oxidoreductase Subunit A12	Protein Coding	2
51	NUBPL	NUBP Iron-Sulfur Cluster Assembly Factor, Mitochondrial	Protein Coding	2
52	DMAC1	Distal Membrane Arm Assembly Component 1	Protein Coding	2
53	NDUFAF2	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 2	Protein Coding	2
54	PYURF	PIGY Upstream Open Reading Frame	Protein Coding	1
55	SFXN4	Sideroflexin 4	Protein Coding	1
56	NDUFA11	NADH:Ubiquinone Oxidoreductase Subunit A11	Protein Coding	1
57	HSCB	HscB Mitochondrial Iron-Sulfur Cluster Cochaperone	Protein Coding	1
58	NDUFAF8	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 8	Protein Coding	1
59	HSPA9	Heat Shock Protein Family A (Hsp70) Member 9	Protein Coding	1
60	NDUFS7	NADH:Ubiquinone Oxidoreductase Core Subunit S7	Protein Coding	1
61	MT-ND3	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 3	Protein Coding	1
62	NDUFA9	NADH:Ubiquinone Oxidoreductase Subunit A9	Protein Coding	1
63	NDUFS8	NADH:Ubiquinone Oxidoreductase Core Subunit S8	Protein Coding	1
64	OXA1L	OXA1L Mitochondrial Inner Membrane Insertase	Protein Coding	1
65	LYRM2	LYR Motif Containing 2	Protein Coding	1
66	NDUFAF5	NADH:Ubiquinone Oxidoreductase Complex Assembly Factor 5	Protein Coding	1
67	TMEM126A	Transmembrane Protein 126A	Protein Coding	1
68	MT-ND4L	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 4L	Protein Coding	1
69	TMEM70	Transmembrane Protein 70	Protein Coding	1
70	ECSIT	ECSIT Signaling Integrator	Protein Coding	1

Disorders associated with Complex I biogenesis SuperPath

according to GeneCards Suite gene sharing

#	Disorder	Type	Genes	Score
1	Leigh syndrome, nuclear	Enrichment	FOXRED1, MT-ND1, MT-ND2, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFA13, NDUFAF2, NDUFAF6, NDUFS4, NDUFV1	11.56
2	Mitochondrial complex i deficiency, nuclear type 1	Enrichment	ACAD9, FOXRED1, MT-ND1, MT-ND2, MT-ND4, NDUFA1, NDUFA13, NDUFA6, NDUFAF1, NDUFAF2, NDUFAF3, NDUFAF4, NDUFB10, NDUFB11, NDUFB3, NDUFB9, NDUFS1, NDUFS2, NDUFS3, NDUFS4, NDUFS6, NDUFV1, NDUFV2, NUBPL, TIMMDC1, TMEM126B	10.99
3	Mitochondrial disease	Enrichment	FOXRED1, MT-ND1, MT-ND2, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFA6, NDUFC2, NDUFS1, NDUFS4, TMEM126B	10.84
4	Leigh syndrome, mitochondrial	Enrichment	MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, MT-ND6	10.80
5	Leber optic atrophy and dystonia	Enrichment	MT-ND1, MT-ND3, MT-ND4, MT-ND5, MT-ND6	10.76
6	Leigh disease	Enrichment	FOXRED1, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, MT-ND6, NDUFA13, NDUFAF2, NDUFAF5, NDUFAF6, NDUFS1, NDUFS4, NDUFS7, NDUFS8, NDUFV1	10.60
7	Leber hereditary optic neuropathy, modifier of	Enrichment	MT-ND1, MT-ND2, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFS2	9.85
8	Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes	Enrichment	MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, MT-ND6, NDUFS1	9.23
9	Leber plus disease	Enrichment	MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND5, MT-ND6, NDUFAF5, NDUFS2	7.70
10	Mitochondrial complex v deficiency, mitochondrial type 1	Enrichment	MT-ND1, MT-ND2, MT-ND4, MT-ND4L, MT-ND5	7.21
11	Neuropathy, ataxia, and retinitis pigmentosa	Enrichment	MT-ND1, MT-ND2, MT-ND4, MT-ND4L, MT-ND5	7.21
12	Striatonigral degeneration, infantile, mitochondrial	Enrichment	MT-ND1, MT-ND2, MT-ND4, MT-ND4L, MT-ND5	7.21
13	Camptodactyly of fingers	Enrichment	MT-ND1, MT-ND2, MT-ND4, MT-ND4L, MT-ND5	7.21
14	Hypertelorism	Enrichment	MT-ND1, MT-ND2, MT-ND4, MT-ND4L, MT-ND5	5.65
15	Mitochondrial myopathy, infantile, transient	Enrichment	MT-ND1, MT-ND2, MT-ND5	5.26
16	Mitochondrial myopathy with reversible cytochrome c oxidase deficiency	Enrichment	MT-ND1, MT-ND2, MT-ND5	5.11
17	Alzheimer disease mitochondrial	Enrichment	MT-ND1, MT-ND2	4.78
18	Mitochondrial complex v deficiency, nuclear type 5	Enrichment	MT-ND5, NDUFV1	4.30
19	Optic atrophy plus syndrome	Enrichment	MT-ND1, MT-ND4, MT-ND6, TMEM126A	4.20
20	Parkinson disease 6, autosomal recessive early-onset	Enrichment	MT-ND5, MT-ND6	3.60
21	Rare mitochondrial non-syndromic sensorineural deafness	Enrichment	MT-ND1, MT-ND4	3.13
22	Lactic acidosis	Enrichment	NDUFA13, NDUFS4	2.97
23	Retinitis pigmentosa	Enrichment	MT-ND1, MT-ND2, MT-ND4, MT-ND4L, MT-ND5	2.75
24	Mitochondrial complex i deficiency, nuclear type 30	Enrichment	NDUFB11	2.38
25	Mitochondrial complex v deficiency, nuclear type 2	Enrichment	TMEM70	2.38
26	Mitochondrial complex i deficiency, nuclear type 37	Enrichment	NDUFA8	2.38
27	Mitochondrial complex i deficiency, nuclear type 9	Enrichment	NDUFS6	2.38
28	Mitochondrial complex i deficiency, nuclear type 23	Enrichment	NDUFA12	2.38
29	Mitochondrial complex i deficiency, nuclear type 25	Enrichment	NDUFB3	2.38
30	Mitochondrial complex i deficiency, nuclear type 31	Enrichment	TIMMDC1	2.38
31	Mitochondrial complex i deficiency, nuclear type 19	Enrichment	FOXRED1	2.38
32	Mitochondrial complex i deficiency, nuclear type 22	Enrichment	NDUFA10	2.38
33	Fanconi renotubular syndrome 5	Enrichment	NDUFAF6	2.38
34	Mitochondrial complex i deficiency, nuclear type 8	Enrichment	NDUFS3	2.38
35	Mitochondrial complex i deficiency, nuclear type 11	Enrichment	NDUFAF1	2.38
36	Mitochondrial complex i deficiency, nuclear type 17	Enrichment	NDUFAF6	2.38
37	Mitochondrial complex i deficiency, nuclear type 29	Enrichment	TMEM126B	2.38
38	Linear skin defects with multiple congenital anomalies 3	Enrichment	NDUFB11	2.38
39	Oncocytoma	Enrichment	MT-ND6	2.38
40	Mitochondrial complex i deficiency, nuclear type 24	Enrichment	NDUFB9	2.38
41	Mitochondrial complex i deficiency, nuclear type 33	Enrichment	NDUFA6	2.38
42	Mitochondrial complex v deficiency	Enrichment	TMEM70	2.38
43	Mitochondrial complex i deficiency, nuclear type 6	Enrichment	NDUFS2	2.38
44	Mitochondrial complex i deficiency, nuclear type 21	Enrichment	NUBPL	2.38
45	Mitochondrial complex i deficiency, nuclear type 32	Enrichment	NDUFB8	2.38
46	Mitochondrial complex i deficiency, nuclear type 39	Enrichment	NDUFB7	2.38
47	Leber-like hereditary optic neuropathy, autosomal recessive 2	Enrichment	NDUFS2	2.38
48	Progressive cavitating leukoencephalopathy	Enrichment	NDUFV2	2.38
49	Mitochondrial disorder due to a defect in assembly or maturation of the respiratory chain complexes	Enrichment	NDUFB7	2.38
50	Mitochondrial complex i deficiency, mitochondrial type 1	Enrichment	MT-ND3	2.31
51	Even-plus syndrome	Enrichment	HSPA9	2.31
52	Anemia, sideroblastic, 4	Enrichment	HSPA9	2.31
53	Mitochondrial complex i deficiency, nuclear type 26	Enrichment	NDUFA9	2.31
54	Optic atrophy 7 with or without auditory neuropathy	Enrichment	TMEM126A	2.31
55	Combined oxidative phosphorylation deficiency 18	Enrichment	SFXN4	2.31
56	Autosomal recessive optic atrophy, opa7 type	Enrichment	TMEM126A	2.31
57	Anemia, sideroblastic, 5	Enrichment	HSCB	2.31
58	Deafness, sensorineural, autosomal-mitochondrial type	Enrichment	MT-ND1	2.08
59	Thyroid carcinoma, hurthle cell	Enrichment	NDUFA13	2.08
60	Leukoencephalopathy, cystic, without megalencephaly	Enrichment	NDUFA2	2.08
61	Mitochondrial complex i deficiency, nuclear type 10	Enrichment	NDUFAF2	2.08
62	Mitochondrial complex i deficiency, nuclear type 15	Enrichment	NDUFAF4	2.08
63	Mitochondrial complex i deficiency, nuclear type 12	Enrichment	NDUFA1	2.08
64	Mitochondrial complex i deficiency, nuclear type 36	Enrichment	NDUFC2	2.08
65	Nuclear type mitochondrial complex i deficiency	Enrichment	NDUFV1	2.08
66	Mitochondrial complex i deficiency, nuclear type 13	Enrichment	NDUFA2	2.08
67	Mitochondrial complex i deficiency, nuclear type 7	Enrichment	NDUFV2	2.08
68	Dystonia	Enrichment	MT-ND1, MT-ND6	2.04
69	Hyperphosphatasia with impaired intellectual development syndrome 6	Enrichment	PYURF	2.01
70	Mitochondrial complex i deficiency, nuclear type 14	Enrichment	NDUFA11	2.01
71	Autosomal recessive sideroblastic anemia	Enrichment	HSPA9	2.01
72	Mitochondrial complex i deficiency, nuclear type 16	Enrichment	NDUFAF5	2.01
73	Linear skin defects with multiple congenital anomalies 1	Enrichment	NDUFB11	1.91
74	Mitochondrial complex i deficiency, nuclear type 20	Enrichment	ACAD9	1.91
75	Uv-sensitive syndrome 2	Enrichment	NDUFAF2	1.91
76	Mitochondrial complex v deficiency, nuclear type 1	Enrichment	NDUFS1	1.91
77	Mitochondrial complex i deficiency, nuclear type 35	Enrichment	NDUFB10	1.91
78	Mitochondrial complex i deficiency, nuclear type 28	Enrichment	NDUFA13	1.91
79	Mitochondrial complex iii deficiency, nuclear type 1	Enrichment	NDUFS7	1.83
80	Mitochondrial myopathy, episodic, with or without optic atrophy and reversible leukoencephalopathy	Enrichment	MT-ND3	1.83
81	Mitochondrial complex i deficiency, nuclear type 34	Enrichment	NDUFAF8	1.83
82	Cardiomyopathy, infantile histiocytoid	Enrichment	NDUFB11	1.78
83	Mitochondrial complex i deficiency, nuclear type 18	Enrichment	NDUFAF3	1.78
84	Primary fanconi renotubular syndrome	Enrichment	NDUFAF6	1.78
85	Mitochondrial complex ii deficiency, nuclear type 1	Enrichment	NDUFS8	1.71
86	Cockayne syndrome a	Enrichment	NDUFAF2	1.69
87	Mitochondrial oxidative phosphorylation disorder	Enrichment	NUBPL	1.69
88	Deafness, nonsyndromic sensorineural, mitochondrial	Enrichment	MT-ND1	1.61
89	Parkinsonism	Enrichment	MT-ND6	1.54
90	Cockayne syndrome	Enrichment	NDUFAF2	1.54
91	Isolated atp synthase deficiency	Enrichment	TMEM70	1.49
92	Optic nerve disease	Enrichment	MT-ND1	1.39
93	Familial colorectal cancer	Enrichment	MT-ND4L	1.39
94	Migraine with or without aura 1	Enrichment	MT-ND1	1.35
95	Myoclonic epilepsy associated with ragged-red fibers	Enrichment	MT-ND5	1.31
96	Restrictive cardiomyopathy	Enrichment	MT-ND1	1.31
97	Alzheimer's disease	Enrichment	MT-ND1	1.28
98	Hydrocephalus, congenital, 1	Enrichment	MT-ND1	1.19
99	Alzheimer disease, familial, 1	Enrichment	MT-ND1	1.17
100	Sudden infant death syndrome	Enrichment	MT-ND1	1.17
101	Parkinson's disease	Enrichment	MT-ND1	1.12
102	Attention deficit-hyperactivity disorder	Enrichment	MT-ND1	1.06
103	Mitochondrial complex iv deficiency, nuclear type 1	Enrichment	NDUFV1	1.06
104	Parkinson disease, late-onset	Enrichment	MT-ND1	1.02
105	Auditory neuropathy	Enrichment	MT-ND6	0.99
106	Differentiated thyroid carcinoma	Enrichment	NDUFA13	0.95
107	Hypertrophic cardiomyopathy	Enrichment	NDUFA13	0.79
108	Rare genetic deafness	Enrichment	MT-ND1	0.55
109	Colorectal cancer	Enrichment	MT-ND1	0.52
110	Hereditary retinal dystrophy	Enrichment	MT-ND4, MT-ND6	0.47
111	Fundus dystrophy	Enrichment	MT-ND4, MT-ND6	0.47

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

Complex I biogenesis

Pathway Network for Complex I biogenesis

Member Pathways for Complex I biogenesis

Pathways in the Complex I biogenesis SuperPath

Associated Genes for Complex I biogenesis

Associated Disorders for Complex I biogenesis

Disorders associated with Complex I biogenesis SuperPath

STRING Interaction Network for Complex I biogenesis

Sources for Complex I biogenesis