Mitochondrial protein degradation

No Pathway Network information available for Mitochondrial protein degradation

Pathways in the Mitochondrial protein degradation SuperPath

#	Name	Source	Genes
1	Mitochondrial protein degradation	Reactome	ACAD8 ACADSB ACAT1 ACO2 ACOT2 AFG3L2 ALAS1 ALDH18A1 ALDH1B1 ALDH2 APP ARG2 ATP5F1A ATP5F1B ATP5F1C ATP5MG ATP5PD ATP5PF ATP5PO BDH1 CHCHD2 CLPP CLPX COX4I1 COX5A COX5B CS DBT DLD ECH1 ECI1 FECH FH GLUD1 HADH HMGCS2 HSD17B10 HSPA9 HSPD1 HTRA2 IARS2 IDH2 IDH3A LDHD LONP1 MDH2 ME2 MICU2 MRPL12 MRPL32 MRPS10 MRPS2 MT-ATP6 MT-CO1 MT-CO2 MT-ND1 MT-ND2 MT-ND5 MT-ND6 NADK2 NDUFA13 NDUFA2 NDUFB6 NDUFS1 NDUFS3 NDUFV1 NDUFV3 OGDH OMA1 OPA1 OXCT1 OXSM PCCB PDHA1 PDHB PDK1 PMPCA PRELID1 PRKACA SHMT2 SMDT1 SPG7 SSBP1 STAR STARD7 SUCLG2 TFAM TIMM10 TIMM17A TIMM22 TIMM9 TRIAP1 TWNK UQCRC2 UQCRQ YME1L1 (see all 96) (see less)

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

#	Symbol	Description	Category	# Related Pathways
1	TIMM17A	Translocase Of Inner Mitochondrial Membrane 17A	Protein Coding	1
2	ATP5PD	ATP Synthase Peripheral Stalk Subunit D	Protein Coding	1
3	ATP5MG	ATP Synthase Membrane Subunit G	Protein Coding	1
4	YME1L1	YME1 Like 1 ATPase	Protein Coding	1
5	CLPX	Caseinolytic Mitochondrial Matrix Peptidase Chaperone Subunit X	Protein Coding	1
6	AFG3L2	AFG3 Like Matrix AAA Peptidase Subunit 2	Protein Coding	1
7	ACOT2	Acyl-CoA Thioesterase 2	Protein Coding	1
8	OMA1	OMA1 Zinc Metallopeptidase	Protein Coding	1
9	COX4I1	Cytochrome C Oxidase Subunit 4I1	Protein Coding	1
10	COX5B	Cytochrome C Oxidase Subunit 5B	Protein Coding	1
11	NADK2	NAD Kinase 2, Mitochondrial	Protein Coding	1
12	CS	Citrate Synthase	Protein Coding	1
13	DBT	Dihydrolipoamide Branched Chain Transacylase E2	Protein Coding	1
14	ECI1	Enoyl-CoA Delta Isomerase 1	Protein Coding	1
15	DLD	Dihydrolipoamide Dehydrogenase	Protein Coding	1
16	ECH1	Enoyl-CoA Hydratase 1	Protein Coding	1
17	LDHD	Lactate Dehydrogenase D	Protein Coding	1
18	ALAS1	5''-Aminolevulinate Synthase 1	Protein Coding	1
19	ALDH2	Aldehyde Dehydrogenase 2 Family Member	Protein Coding	1
20	ALDH1B1	Aldehyde Dehydrogenase 1 Family Member B1	Protein Coding	1
21	MICU2	Mitochondrial Calcium Uptake 2	Protein Coding	1
22	FECH	Ferrochelatase	Protein Coding	1
23	FH	Fumarate Hydratase	Protein Coding	1
24	PMPCA	Peptidase, Mitochondrial Processing Subunit Alpha	Protein Coding	1
25	TIMM10	Translocase Of Inner Mitochondrial Membrane 10	Protein Coding	1
26	TIMM9	Translocase Of Inner Mitochondrial Membrane 9	Protein Coding	1
27	ACAD8	Acyl-CoA Dehydrogenase Family Member 8	Protein Coding	1
28	UQCRQ	Ubiquinol-Cytochrome C Reductase Complex III Subunit VII	Protein Coding	1
29	PRELID1	PRELI Domain Containing 1	Protein Coding	1
30	HTRA2	HtrA Serine Peptidase 2	Protein Coding	1
31	GLUD1	Glutamate Dehydrogenase 1	Protein Coding	1
32	TIMM22	Translocase Of Inner Mitochondrial Membrane 22	Protein Coding	1
33	HSD17B10	Hydroxysteroid 17-Beta Dehydrogenase 10	Protein Coding	1
34	HADH	Hydroxyacyl-CoA Dehydrogenase	Protein Coding	1
35	HMGCS2	3-Hydroxy-3-Methylglutaryl-CoA Synthase 2	Protein Coding	1
36	HSPA9	Heat Shock Protein Family A (Hsp70) Member 9	Protein Coding	1
37	HSPD1	Heat Shock Protein Family D (Hsp60) Member 1	Protein Coding	1
38	IDH2	Isocitrate Dehydrogenase (NADP(+)) 2	Protein Coding	1
39	IDH3A	Isocitrate Dehydrogenase (NAD(+)) 3 Catalytic Subunit Alpha	Protein Coding	1
40	APP	Amyloid Beta Precursor Protein	Protein Coding	1
41	ACADSB	Acyl-CoA Dehydrogenase Short/Branched Chain	Protein Coding	1
42	ACAT1	Acetyl-CoA Acetyltransferase 1	Protein Coding	1
43	ARG2	Arginase 2	Protein Coding	1
44	MDH2	Malate Dehydrogenase 2	Protein Coding	1
45	ME2	Malic Enzyme 2	Protein Coding	1
46	MT-ATP6	Mitochondrially Encoded ATP Synthase Membrane Subunit 6	Protein Coding	1
47	MT-CO1	Mitochondrially Encoded Cytochrome C Oxidase I	Protein Coding	1
48	MT-CO2	Mitochondrially Encoded Cytochrome C Oxidase II	Protein Coding	1
49	MT-ND1	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 1	Protein Coding	1
50	MT-ND2	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 2	Protein Coding	1
51	MT-ND5	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 5	Protein Coding	1
52	MT-ND6	Mitochondrially Encoded NADH:Ubiquinone Oxidoreductase Core Subunit 6	Protein Coding	1
53	NDUFA2	NADH:Ubiquinone Oxidoreductase Subunit A2	Protein Coding	1
54	NDUFB6	NADH:Ubiquinone Oxidoreductase Subunit B6	Protein Coding	1
55	NDUFS1	NADH:Ubiquinone Oxidoreductase Core Subunit S1	Protein Coding	1
56	NDUFS3	NADH:Ubiquinone Oxidoreductase Core Subunit S3	Protein Coding	1
57	NDUFV1	NADH:Ubiquinone Oxidoreductase Core Subunit V1	Protein Coding	1
58	NDUFV3	NADH:Ubiquinone Oxidoreductase Subunit V3	Protein Coding	1
59	OGDH	Oxoglutarate Dehydrogenase	Protein Coding	1
60	OPA1	OPA1 Mitochondrial Dynamin Like GTPase	Protein Coding	1
61	ATP5F1A	ATP Synthase F1 Subunit Alpha	Protein Coding	1
62	ACO2	Aconitase 2	Protein Coding	1
63	OXCT1	3-Oxoacid CoA-Transferase 1	Protein Coding	1
64	ATP5F1B	ATP Synthase F1 Subunit Beta	Protein Coding	1
65	ATP5F1C	ATP Synthase F1 Subunit Gamma	Protein Coding	1
66	PCCB	Propionyl-CoA Carboxylase Subunit Beta	Protein Coding	1
67	NDUFA13	NADH:Ubiquinone Oxidoreductase Subunit A13	Protein Coding	1
68	MRPS2	Mitochondrial Ribosomal Protein S2	Protein Coding	1
69	CHCHD2	Coiled-Coil-Helix-Coiled-Coil-Helix Domain Containing 2	Protein Coding	1
70	TRIAP1	TP53 Regulated Inhibitor Of Apoptosis 1	Protein Coding	1
71	PDHA1	Pyruvate Dehydrogenase E1 Subunit Alpha 1	Protein Coding	1
72	PDHB	Pyruvate Dehydrogenase E1 Subunit Beta	Protein Coding	1
73	PDK1	Pyruvate Dehydrogenase Kinase 1	Protein Coding	1
74	ATP5PF	ATP Synthase Peripheral Stalk Subunit F6	Protein Coding	1
75	ATP5PO	ATP Synthase Peripheral Stalk Subunit OSCP	Protein Coding	1
76	OXSM	3-Oxoacyl-ACP Synthase, Mitochondrial	Protein Coding	1
77	MRPS10	Mitochondrial Ribosomal Protein S10	Protein Coding	1
78	PRKACA	Protein Kinase CAMP-Activated Catalytic Subunit Alpha	Protein Coding	1
79	IARS2	Isoleucyl-TRNA Synthetase 2, Mitochondrial	Protein Coding	1
80	TWNK	Twinkle MtDNA Helicase	Protein Coding	1
81	STARD7	StAR Related Lipid Transfer Domain Containing 7	Protein Coding	1
82	ALDH18A1	Aldehyde Dehydrogenase 18 Family Member A1	Protein Coding	1
83	MRPL12	Mitochondrial Ribosomal Protein L12	Protein Coding	1
84	BDH1	3-Hydroxybutyrate Dehydrogenase 1	Protein Coding	1
85	SHMT2	Serine Hydroxymethyltransferase 2	Protein Coding	1
86	MRPL32	Mitochondrial Ribosomal Protein L32	Protein Coding	1
87	SPG7	SPG7 Matrix AAA Peptidase Subunit, Paraplegin	Protein Coding	1
88	SSBP1	Single Stranded DNA Binding Protein 1	Protein Coding	1
89	STAR	Steroidogenic Acute Regulatory Protein	Protein Coding	1
90	TFAM	Transcription Factor A, Mitochondrial	Protein Coding	1
91	UQCRC2	Ubiquinol-Cytochrome C Reductase Core Protein 2	Protein Coding	1
92	CLPP	Caseinolytic Mitochondrial Matrix Peptidase Proteolytic Subunit	Protein Coding	1
93	SUCLG2	Succinate-CoA Ligase GDP-Forming Subunit Beta	Protein Coding	1
94	SMDT1	Single-Pass Membrane Protein With Aspartate Rich Tail 1	Protein Coding	1
95	LONP1	Lon Peptidase 1, Mitochondrial	Protein Coding	1
96	COX5A	Cytochrome C Oxidase Subunit 5A	Protein Coding	1

Disorders associated with Mitochondrial protein degradation SuperPath

according to GeneCards Suite gene sharing

#	Disorder	Type	Genes	Score
1	Leigh syndrome, nuclear	Enrichment	ATP5PO, IARS2, MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5, MT-ND6, NDUFA13, NDUFV1	10.84
2	Mitochondrial myopathy with reversible cytochrome c oxidase deficiency	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5	10.78
3	Mitochondrial myopathy, infantile, transient	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5	10.64
4	Mitochondrial disease	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5, MT-ND6, NDUFS1, OPA1, SPG7, TWNK, UQCRC2	10.61
5	Leigh disease	Enrichment	ATP5PO, IARS2, MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5, MT-ND6, NDUFA13, NDUFS1, NDUFV1	10.55
6	Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5, MT-ND6, NDUFS1	9.67
7	Leber hereditary optic neuropathy, modifier of	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5, MT-ND6	8.21
8	Leigh syndrome, mitochondrial	Enrichment	MT-ATP6, MT-ND1, MT-ND2, MT-ND5, MT-ND6	8.15
9	Mitochondrial complex v deficiency, mitochondrial type 1	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5	7.66
10	Neuropathy, ataxia, and retinitis pigmentosa	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5	7.66
11	Striatonigral degeneration, infantile, mitochondrial	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5	7.66
12	Camptodactyly of fingers	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5	7.66
13	Optic atrophy plus syndrome	Enrichment	ACO2, AFG3L2, MT-ND1, MT-ND6, OPA1, PMPCA, SPG7	7.66
14	Mitochondrial complex i deficiency, nuclear type 1	Enrichment	MT-ND1, MT-ND2, NDUFA13, NDUFS1, NDUFS3, NDUFV1	6.61
15	Hypertelorism	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5	5.77
16	Cox deficiency, benign infantile mitochondrial myopathy	Enrichment	COX4I1, COX5A, MT-CO1, MT-CO2	5.65
17	Leber optic atrophy and dystonia	Enrichment	MT-ND1, MT-ND5, MT-ND6	5.47
18	Leber plus disease	Enrichment	MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5, MT-ND6	5.32
19	Mitochondrial complex iv deficiency, nuclear type 1	Enrichment	COX4I1, MT-CO1, MT-CO2, NDUFV1	4.81
20	Alzheimer disease mitochondrial	Enrichment	MT-ND1, MT-ND2	4.30
21	Lactic acidosis	Enrichment	ATP5F1A, DLD, NDUFA13	4.14
22	Mitochondrial complex v deficiency, nuclear type 5	Enrichment	MT-ND5, NDUFV1	3.83
23	Pyruvate dehydrogenase e1-alpha deficiency	Enrichment	LONP1, PDHA1	3.53
24	Autosomal recessive isolated optic atrophy	Enrichment	ACO2, YME1L1	3.53
25	Hyperinsulinemic hypoglycemia, familial, 1	Enrichment	GLUD1, HADH	3.14
26	Deafness, nonsyndromic sensorineural, mitochondrial	Enrichment	MT-CO1, MT-ND1	3.14
27	Parkinson disease 6, autosomal recessive early-onset	Enrichment	MT-ND5, MT-ND6	3.14
28	Nonsyndromic genetic hyperinsulinism	Enrichment	GLUD1, HADH	3.14
29	Retinitis pigmentosa	Enrichment	IDH3A, MT-ATP6, MT-CO1, MT-CO2, MT-ND1, MT-ND2, MT-ND5	3.12
30	Isolated atp synthase deficiency	Enrichment	ATP5F1A, MT-ATP6	2.87
31	Perrault syndrome 2	Enrichment	CLPP, TWNK	2.76
32	Dystonia	Enrichment	AFG3L2, MT-ND1, MT-ND6	2.72
33	Optic nerve disease	Enrichment	MT-ATP6, MT-ND1	2.67
34	Familial colorectal cancer	Enrichment	MT-CO1, MT-CO2	2.67
35	Rare mitochondrial non-syndromic sensorineural deafness	Enrichment	MT-CO1, MT-ND1	2.67
36	Isolated complex iii deficiency	Enrichment	UQCRC2, UQCRQ	2.58
37	Alzheimer's disease	Enrichment	APP, MT-ND1	2.43
38	Perrault syndrome 1	Enrichment	CLPP, TWNK	2.25
39	Alzheimer disease, familial, 1	Enrichment	APP, MT-ND1	2.20
40	Sudden infant death syndrome	Enrichment	MT-ND1, PDHA1	2.20
41	Hereditary leiomyomatosis and renal cell cancer	Enrichment	FH	2.15
42	3-hydroxyacyl-coa dehydrogenase deficiency	Enrichment	HADH	2.15
43	D-lactic aciduria with gout	Enrichment	LDHD	2.15
44	Oxoglutarate dehydrogenase deficiency	Enrichment	OGDH	2.15
45	Hsd10 mitochondrial disease	Enrichment	HSD17B10	2.15
46	Ataxia and polyneuropathy, adult-onset	Enrichment	MT-ATP6	2.15
47	Spastic paraplegia 9a, autosomal dominant	Enrichment	ALDH18A1	2.15
48	Epilepsy, familial adult myoclonic, 2	Enrichment	STARD7	2.15
49	Myoglobinuria, recurrent	Enrichment	MT-CO1	2.15
50	Codas syndrome	Enrichment	LONP1	2.15
51	Myopathy, lactic acidosis, and sideroblastic anemia 3	Enrichment	MT-ATP6	2.15
52	Parkinson disease 13, autosomal dominant	Enrichment	HTRA2	2.15
53	Fumarase deficiency	Enrichment	FH	2.15
54	Isobutyryl-coa dehydrogenase deficiency	Enrichment	ACAD8	2.15
55	2-methylbutyryl-coa dehydrogenase deficiency	Enrichment	ACADSB	2.15
56	Optic atrophy 13 with retinal and foveal abnormalities	Enrichment	SSBP1	2.15
57	Spastic ataxia 5, autosomal recessive	Enrichment	AFG3L2	2.15
58	2,4-dienoyl-coa reductase deficiency	Enrichment	NADK2	2.15
59	Succinyl-coa:3-oxoacid-coa transferase deficiency	Enrichment	OXCT1	2.15
60	Cutis laxa, autosomal recessive, type iiia	Enrichment	ALDH18A1	2.15
61	Even-plus syndrome	Enrichment	HSPA9	2.15
62	Anemia, sideroblastic, 4	Enrichment	HSPA9	2.15
63	Retinitis pigmentosa 90	Enrichment	IDH3A	2.15
64	Spastic paraplegia 9b, autosomal recessive	Enrichment	ALDH18A1	2.15
65	Maple syrup urine disease, type ii	Enrichment	DBT	2.15
66	Mitochondrial complex i deficiency, nuclear type 8	Enrichment	NDUFS3	2.15
67	Progressive external ophthalmoplegia with mitochondrial dna deletions, autosomal dominant 3	Enrichment	TWNK	2.15
68	Mitochondrial complex v deficiency, nuclear type 4b	Enrichment	ATP5F1A	2.15
69	Oncocytoma	Enrichment	MT-ND6	2.15
70	Syndromic x-linked intellectual disability type 10	Enrichment	HSD17B10	2.15
71	Spinocerebellar ataxia 28	Enrichment	AFG3L2	2.15
72	Cutis laxa, autosomal dominant 3	Enrichment	ALDH18A1	2.15
73	Alcohol sensitivity, acute	Enrichment	ALDH2	2.15
74	Perrault syndrome 5	Enrichment	TWNK	2.15
75	Leiomyoma cutis	Enrichment	FH	2.15
76	Hyperinsulinemic hypoglycemia, familial, 6	Enrichment	GLUD1	2.15
77	Gemistocytic astrocytoma	Enrichment	IDH2	2.15
78	Protoplasmic astrocytoma	Enrichment	IDH2	2.15
79	Cerebral amyloid angiopathy, app-related	Enrichment	APP	2.15
80	Spastic paraplegia 13, autosomal dominant	Enrichment	HSPD1	2.15
81	D-2-hydroxyglutaric aciduria 2	Enrichment	IDH2	2.15
82	Cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia	Enrichment	IARS2	2.15
83	Dominant hereditary optic atrophy	Enrichment	OPA1	2.15
84	Neurodevelopmental disorder with cardiomyopathy, spasticity, and brain abnormalities	Enrichment	SHMT2	2.15
85	Spastic ataxia 5	Enrichment	AFG3L2	2.15
86	Pigmented nodular adrenocortical disease, primary, 4	Enrichment	PRKACA	2.15
87	Autosomal recessive cutis laxa type iii	Enrichment	ALDH18A1	2.15
88	Combined oxidative phosphorylation deficiency 22	Enrichment	ATP5F1A	2.15
89	Developmental and epileptic encephalopathy 51	Enrichment	MDH2	2.15
90	Parkinson disease 22, autosomal dominant	Enrichment	CHCHD2	2.15
91	Protoporphyria, erythropoietic, 2	Enrichment	CLPX	2.15
92	Fumarate hydratase deficiency	Enrichment	FH	2.15
93	Mixed oligodendroglioma-astrocytoma	Enrichment	IDH2	2.15
94	Optic atrophy 11	Enrichment	YME1L1	2.15
95	Cardioacrofacial dysplasia 1	Enrichment	PRKACA	2.15
96	Anaplastic oligoastrocytoma	Enrichment	IDH2	2.15
97	Mitochondrial complex iv deficiency, nuclear type 20	Enrichment	COX5A	2.15
98	Mitochondrial complex v deficiency, nuclear type 4a	Enrichment	ATP5F1A	2.15
99	Combined oxidative phosphorylation deficiency 43	Enrichment	TIMM22	2.15
100	Combined oxidative phosphorylation deficiency 45	Enrichment	MRPL12	2.15
101	Mitochondrial complex iv deficiency, nuclear type 16	Enrichment	COX4I1	2.15
102	Hypermetabolism due to uncoupled mitochondrial oxidative phosphorylation 2	Enrichment	ATP5F1B	2.15
103	Mitochondrial dna depletion syndrome, hepatocerebrorenal form	Enrichment	TWNK	2.15
104	Fibrillary astrocytoma	Enrichment	IDH2	2.15
105	Acute myocardial infarction	Enrichment	IDH2	2.15
106	Lateral sclerosis	Enrichment	SPG7	2.15
107	Non-classic congenital lipoid adrenal hyperplasia due to star deficency	Enrichment	STAR	2.15
108	Maternally-inherited spastic paraplegia	Enrichment	MT-ATP6	2.15
109	Classic congenital lipoid adrenal hyperplasia due to star deficency	Enrichment	STAR	2.15
110	Autosomal dominant complex spastic paraplegia type 9b	Enrichment	ALDH18A1	2.15
111	Hereditary paraganglioma-pheochromocytoma syndromes	Enrichment	FH, MDH2	2.02
112	Charcot-marie-tooth disease, demyelinating, type 1a	Enrichment	MT-ATP6	1.85
113	Leiomyoma, uterine	Enrichment	FH	1.85
114	Cerebral amyloid angiopathy, cst3-related	Enrichment	APP	1.85
115	Optic atrophy 1	Enrichment	OPA1	1.85
116	Behr syndrome	Enrichment	OPA1	1.85
117	Alpha-methylacetoacetic aciduria	Enrichment	ACAT1	1.85
118	Deafness, sensorineural, autosomal-mitochondrial type	Enrichment	MT-ND1	1.85
119	Hydrocephalus, normal-pressure, 1	Enrichment	PMPCA	1.85
120	Dihydrolipoamide dehydrogenase deficiency	Enrichment	DLD	1.85
121	Cardiomyopathy, infantile hypertrophic	Enrichment	MT-ATP6	1.85
122	Toe syndactyly, telecanthus, and anogenital and renal malformations	Enrichment	STAR	1.85
123	Leukodystrophy, hypomyelinating, 4	Enrichment	HSPD1	1.85
124	Porphyria, acute intermittent	Enrichment	ACO2	1.85
125	Thyroid carcinoma, hurthle cell	Enrichment	NDUFA13	1.85
126	Hyperinsulinemic hypoglycemia, familial, 4	Enrichment	HADH	1.85
127	Protoporphyria, erythropoietic, 1	Enrichment	FECH	1.85
128	Leukoencephalopathy, cystic, without megalencephaly	Enrichment	NDUFA2	1.85
129	Methemoglobinemia due to deficiency of methemoglobin reductase	Enrichment	DLD	1.85
130	Mitochondrial dna depletion syndrome 14	Enrichment	OPA1	1.85
131	Optic atrophy 8	Enrichment	OPA1	1.85
132	Pyruvate dehydrogenase e1-beta deficiency	Enrichment	PDHB	1.85
133	Glycogen storage disease iii	Enrichment	AFG3L2	1.85
134	Spastic paraplegia 79b, autosomal recessive	Enrichment	SPG7	1.85
135	Amed syndrome, digenic	Enrichment	ALDH2	1.85
136	Optic atrophy 12	Enrichment	AFG3L2	1.85
137	Frontotemporal dementia and/or amyotrophic lateral sclerosis 2	Enrichment	SPG7	1.85
138	Spastic paraplegia 5a, autosomal recessive	Enrichment	ALDH18A1	1.85
139	Combined oxidative phosphorylation deficiency 36	Enrichment	MRPS2	1.85
140	Perrault syndrome 3	Enrichment	CLPP	1.85
141	3-hydroxy-3-methylglutaryl-coa synthase-2 deficiency	Enrichment	HMGCS2	1.85
142	Charcot-marie-tooth disease type 1a	Enrichment	MT-ATP6	1.85
143	Oligodendroglioma	Enrichment	IDH2	1.85
144	Depressive disorder	Enrichment	TWNK	1.85
145	Fibrolamellar carcinoma	Enrichment	PRKACA	1.85
146	Acute porphyria	Enrichment	ACO2	1.85
147	Autosomal recessive sideroblastic anemia	Enrichment	HSPA9	1.85
148	Anaplastic oligodendroglioma	Enrichment	IDH2	1.85
149	Spinocerebellar ataxia 45	Enrichment	FH	1.85
150	Mitochondrial dna depletion syndrome 15	Enrichment	TFAM	1.85
151	Nuclear type mitochondrial complex i deficiency	Enrichment	NDUFV1	1.85
152	Mitochondrial complex i deficiency, nuclear type 13	Enrichment	NDUFA2	1.85
153	Normal pressure hydrocephalus	Enrichment	PMPCA	1.85
154	D-2-hydroxyglutaric aciduria	Enrichment	IDH2	1.85
155	Autosomal erythropoietic protoporphyria	Enrichment	FECH	1.85
156	Erythropoietic protoporphyria, autosomal recessive	Enrichment	FECH	1.85
157	Periodic paralysis with later-onset distal motor neuropathy	Enrichment	MT-ATP6	1.85
158	Tetralogy of fallot	Enrichment	MT-CO1, MT-CO2	1.84
159	Auditory neuropathy	Enrichment	MT-ND6, OPA1	1.84
160	Keratoderma, palmoplantar, with deafness	Enrichment	MT-CO1	1.68
161	Maple syrup urine disease, type ia	Enrichment	DBT	1.68
162	Mitochondrial complex iv deficiency, nuclear type 5	Enrichment	ATP5F1A	1.68
163	Spinocerebellar ataxia, autosomal recessive 2	Enrichment	PMPCA	1.68
164	Mitochondrial dna depletion syndrome 7	Enrichment	TWNK	1.68
165	Spastic paraplegia 7, autosomal recessive	Enrichment	SPG7	1.68
166	Glaucoma, normal tension	Enrichment	OPA1	1.68
167	Infantile cerebellar-retinal degeneration	Enrichment	ACO2	1.68
168	Optic atrophy 9	Enrichment	ACO2	1.68
169	Mitochondrial complex v deficiency, nuclear type 7	Enrichment	ATP5PO	1.68
170	Chronic progressive external ophthalmoplegia	Enrichment	TWNK	1.68
171	Autosomal dominant cutis laxa	Enrichment	ALDH18A1	1.68
172	Hyperinsulinemic hypoglycemia	Enrichment	HADH	1.68
173	Cerebellar disease	Enrichment	AFG3L2	1.68
174	Anaplastic astrocytoma	Enrichment	IDH2	1.68
175	Mitochondrial complex v deficiency, nuclear type 1	Enrichment	NDUFS1	1.68
176	Gonadal dysgenesis	Enrichment	MT-ATP6	1.68
177	3-methylglutaconic aciduria	Enrichment	YME1L1	1.68
178	Mitochondrial complex i deficiency, nuclear type 28	Enrichment	NDUFA13	1.68
179	Myelodysplastic syndrome with ring sideroblasts	Enrichment	MT-CO1	1.68
180	Autosomal dominant optic atrophy, classic form	Enrichment	OPA1	1.68
181	Intermittent maple syrup urine disease	Enrichment	DBT	1.68
182	Classic maple syrup urine disease	Enrichment	DBT	1.68
183	Cardiomyopathy, infantile histiocytoid	Enrichment	MT-ATP6	1.55
184	Propionic acidemia	Enrichment	PCCB	1.55
185	Mitochondrial complex v deficiency, nuclear type 3	Enrichment	UQCRC2	1.55
186	Hereditary recurrent myoglobinuria	Enrichment	MT-CO1	1.55
187	3-methylglutaconic aciduria, type viii	Enrichment	HTRA2	1.55
188	Intermediate maple syrup urine disease	Enrichment	DBT	1.55
189	Familial infantile bilateral striatal necrosis	Enrichment	MT-ATP6	1.55
190	Enchondromatosis, multiple, ollier type	Enrichment	IDH2	1.46
191	3-methylglutaconic aciduria, type iii	Enrichment	OPA1	1.46
192	Sensory ataxic neuropathy, dysarthria, and ophthalmoparesis	Enrichment	TWNK	1.46
193	Mitochondrial complex iii deficiency, nuclear type 4	Enrichment	UQCRQ	1.46
194	Polyneuropathy	Enrichment	SPG7	1.46
195	Familial glucocorticoid deficiency	Enrichment	STAR	1.46
196	Hereditary spastic paraplegia	Enrichment	ALDH18A1, SPG7	1.45
197	Hypertrophic cardiomyopathy	Enrichment	NDUFA13, PMPCA	1.43
198	Alcohol dependence	Enrichment	ALDH2	1.38
199	Progressive external ophthalmoplegia with mitochondrial dna deletions, autosomal dominant 1	Enrichment	TWNK	1.38
200	Multiple enchondromatosis, maffucci type	Enrichment	IDH2	1.31
201	Third-degree atrioventricular block	Enrichment	TWNK	1.31
202	Alzheimer's disease 1	Enrichment	APP	1.31
203	Parkinsonism	Enrichment	MT-ND6	1.31
204	Early-onset autosomal dominant alzheimer disease	Enrichment	APP	1.31
205	Spastic ataxia	Enrichment	AFG3L2, SPG7	1.27
206	Hereditary retinal dystrophy	Enrichment	ACO2, IDH3A, MT-ND6, OPA1, SPG7	1.26
207	Fundus dystrophy	Enrichment	ACO2, IDH3A, MT-ND6, OPA1, SPG7	1.26
208	Glioma susceptibility 1	Enrichment	IDH2	1.26
209	Choreatic disease	Enrichment	AFG3L2	1.26
210	Ellis-van creveld syndrome	Enrichment	PRKACA	1.21
211	Autosomal dominant cerebellar ataxia	Enrichment	OPA1	1.21
212	Migraine with or without aura 1	Enrichment	MT-ND1	1.12
213	Myoclonic epilepsy associated with ragged-red fibers	Enrichment	MT-ND5	1.09
214	Early-onset parkinson's disease	Enrichment	HTRA2	1.09
215	Restrictive cardiomyopathy	Enrichment	MT-ND1	1.09
216	Aortic valve disease 1	Enrichment	MT-ATP6	1.05
217	Diaphragmatic hernia, congenital	Enrichment	LONP1	1.05
218	Lipoid congenital adrenal hyperplasia	Enrichment	STAR	1.02
219	Cataract	Enrichment	IARS2	1.02
220	Hereditary chronic pancreatitis	Enrichment	SPG7	1.02
221	Isolated macular dystrophy	Enrichment	ACO2	1.02
222	Hydrocephalus, congenital, 1	Enrichment	MT-ND1	0.97
223	Pancreatitis, hereditary	Enrichment	SPG7	0.94
224	Colorectal cancer	Enrichment	MT-CO1, MT-ND1	0.90
225	Parkinson's disease	Enrichment	MT-ND1	0.90
226	Leukodystrophy	Enrichment	HSPD1	0.88
227	Centronuclear myopathy	Enrichment	OPA1	0.86
228	Hepatocellular carcinoma	Enrichment	FH	0.84
229	Attention deficit-hyperactivity disorder	Enrichment	MT-ND1	0.84
230	Parkinson disease, late-onset	Enrichment	MT-ND1	0.80
231	Developmental and epileptic encephalopathy 1	Enrichment	MDH2	0.79
232	Differentiated thyroid carcinoma	Enrichment	NDUFA13	0.73
233	Stargardt disease 1	Enrichment	OPA1	0.72
234	Peripheral nervous system disease	Enrichment	IARS2	0.69
235	Neuropathy	Enrichment	IARS2	0.69
236	Leukemia, acute myeloid	Enrichment	IDH2	0.61
237	Charcot-marie-tooth disease	Enrichment	MT-ATP6	0.60
238	West syndrome	Enrichment	MDH2	0.57
239	Sensorineural hearing loss	Enrichment	AFG3L2	0.54
240	Cone-rod dystrophy 2	Enrichment	SSBP1	0.42
241	Rare genetic deafness	Enrichment	MT-ND1	0.37
242	Ovarian cancer	Enrichment	FH	0.29
243	Congenital nervous system abnormality	Enrichment	ALDH18A1	0.28
244	Nervous system disease	Enrichment	ALDH18A1	0.28
245	Microcephaly	Enrichment	ATP5PO	0.23
246	Inherited cancer-predisposing syndrome	Enrichment	FH	0.21

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

Mitochondrial protein degradation

Pathway Network for Mitochondrial protein degradation

Member Pathways for Mitochondrial protein degradation

Pathways in the Mitochondrial protein degradation SuperPath

Associated Genes for Mitochondrial protein degradation

Associated Disorders for Mitochondrial protein degradation

Disorders associated with Mitochondrial protein degradation SuperPath

STRING Interaction Network for Mitochondrial protein degradation

Sources for Mitochondrial protein degradation