Citric acid cycle (TCA cycle)

Pathway network for the Citric acid cycle (TCA cycle) SuperPath

Sources:

Reactome
PubChem
WikiPathways

Pathways in the Citric acid cycle (TCA cycle) SuperPath

#	Name	Source	Genes
1	Citric acid cycle (TCA cycle)	Reactome	ACAT1 ACO2 CS CSKMT DLD DLST FH FXN IDH2 IDH3A IDH3B IDH3G ISCA1 ISCA2 ISCU KGD4 LYRM4 MDH2 NFS1 NNT OGDH SDHA SDHAF1 SDHAF2 SDHAF3 SDHAF4 SDHB SDHC SDHD SIRT3 SUCLA2 SUCLG1 SUCLG2 TRAP1 (see all 34) (see less)
2	Maturation of TCA enzymes and regulation of TCA cycle	Reactome	ACAT1 ACO2 CS CSKMT FXN IDH2 ISCA1 ISCA2 ISCU LYRM4 NFS1 SDHA SDHAF1 SDHAF2 SDHAF3 SDHAF4 SDHB SDHC SDHD SIRT3 (see all 20) (see less)
3	TCA cycle	PubChem	ACO2 CS DLST FH IDH3A IDH3B IDH3G MDH1 MDH2 OGDH SDHA SDHB SDHC SDHD SUCLA2 SUCLG1 SUCLG2 (see all 17) (see less)
4	TCA cycle (aka Krebs or citric acid cycle)	WikiPathways	ACO2 CS DLD DLST FH IDH2 IDH3A IDH3B IDH3G MDH2 OGDH SDHA SDHB SDHC SDHD SUCLG1 SUCLG2 (see all 17) (see less)
5	TCA cycle III (animals)	PubChem	ACO2 CS DLST FH IDH3A IDH3B IDH3G MDH1 MDH2 OGDH SDHA SDHB SDHC SDHD SUCLA2 SUCLG1 SUCLG2 (see all 17) (see less)
6	TCA cycle and deficiency of pyruvate dehydrogenase complex (PDHc)	WikiPathways	ACLY ACO1 CS DLAT DLD DLST FH IDH1 IDH3A MDH1 OGDH PC PCK1 PDHA1 SDHA SUCLG2 (see all 16) (see less)
7	Mitochondrial complex II assembly	WikiPathways	SDHA SDHAF1 SDHAF2 SDHAF3 SDHAF4 SDHB SDHC SDHD (see all 8) (see less)
8	aerobic respiration I (cytochrome c)	PubChem	SDHA SDHB SDHC SDHD
9	NADPH regeneration	Reactome	ACO1 IDH1
10	Abnormal conversion of 2-oxoglutarate to 2-hydroxyglutarate	Reactome	IDH1

Gene overlap in member pathways for Citric acid cycle (TCA cycle) SuperPath

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

#	Symbol	Description	Category	# Related Pathways
1	SDHA	Succinate Dehydrogenase Complex Flavoprotein Subunit A	Protein Coding	8
2	SDHB	Succinate Dehydrogenase Complex Iron Sulfur Subunit B	Protein Coding	7
3	SDHC	Succinate Dehydrogenase Complex Subunit C	Protein Coding	7
4	SDHD	Succinate Dehydrogenase Complex Subunit D	Protein Coding	7
5	CS	Citrate Synthase	Protein Coding	6
6	DLST	Dihydrolipoamide S-Succinyltransferase	Protein Coding	5
7	FH	Fumarate Hydratase	Protein Coding	5
8	IDH3A	Isocitrate Dehydrogenase (NAD(+)) 3 Catalytic Subunit Alpha	Protein Coding	5
9	OGDH	Oxoglutarate Dehydrogenase	Protein Coding	5
10	ACO2	Aconitase 2	Protein Coding	5
11	SUCLG2	Succinate-CoA Ligase GDP-Forming Subunit Beta	Protein Coding	5
12	IDH3B	Isocitrate Dehydrogenase (NAD(+)) 3 Non-Catalytic Subunit Beta	Protein Coding	4
13	IDH3G	Isocitrate Dehydrogenase (NAD(+)) 3 Non-Catalytic Subunit Gamma	Protein Coding	4
14	MDH2	Malate Dehydrogenase 2	Protein Coding	4
15	SUCLG1	Succinate-CoA Ligase GDP/ADP-Forming Subunit Alpha	Protein Coding	4
16	SDHAF4	Succinate Dehydrogenase Complex Assembly Factor 4	Protein Coding	3
17	DLD	Dihydrolipoamide Dehydrogenase	Protein Coding	3
18	IDH2	Isocitrate Dehydrogenase (NADP(+)) 2	Protein Coding	3
19	SDHAF2	Succinate Dehydrogenase Complex Assembly Factor 2	Protein Coding	3
20	SDHAF3	Succinate Dehydrogenase Complex Assembly Factor 3	Protein Coding	3
21	SDHAF1	Succinate Dehydrogenase Complex Assembly Factor 1	Protein Coding	3
22	SUCLA2	Succinate-CoA Ligase ADP-Forming Subunit Beta	Protein Coding	3
23	MDH1	Malate Dehydrogenase 1	Protein Coding	3
24	IDH1	Isocitrate Dehydrogenase (NADP(+)) 1	Protein Coding	3
25	ISCA2	Iron-Sulfur Cluster Assembly 2	Protein Coding	2
26	SIRT3	Sirtuin 3	Protein Coding	2
27	ISCU	Iron-Sulfur Cluster Assembly Enzyme	Protein Coding	2
28	FXN	Frataxin	Protein Coding	2
29	ACAT1	Acetyl-CoA Acetyltransferase 1	Protein Coding	2
30	LYRM4	LYR Motif Containing 4	Protein Coding	2
31	CSKMT	Citrate Synthase Lysine Methyltransferase	Protein Coding	2
32	ISCA1	Iron-Sulfur Cluster Assembly 1	Protein Coding	2
33	NFS1	NFS1 Cysteine Desulfurase	Protein Coding	2
34	ACO1	Aconitase 1	Protein Coding	2
35	TRAP1	TNF Receptor Associated Protein 1	Protein Coding	1
36	NNT	Nicotinamide Nucleotide Transhydrogenase	Protein Coding	1
37	KGD4	Alpha-Ketoglutarate Dehydrogenase Subunit 4	Protein Coding	1
38	PDHA1	Pyruvate Dehydrogenase E1 Subunit Alpha 1	Protein Coding	1
39	PCK1	Phosphoenolpyruvate Carboxykinase 1	Protein Coding	1
40	PC	Pyruvate Carboxylase	Protein Coding	1
41	ACLY	ATP Citrate Lyase	Protein Coding	1
42	DLAT	Dihydrolipoamide S-Acetyltransferase	Protein Coding	1

Disorders associated with Citric acid cycle (TCA cycle) SuperPath

according to GeneCards Suite gene sharing

#	Disorder	Type	Genes	Score
1	Glioblastoma	Direct
2	Hereditary paraganglioma-pheochromocytoma syndromes	Enrichment	DLST, FH, MDH2, SDHA, SDHB, SDHC, SDHD	16.00
3	Mitochondrial complex ii deficiency	Enrichment	SDHA, SDHAF1, SDHB, SDHD	11.19
4	Carney triad	Enrichment	SDHA, SDHB, SDHC	10.83
5	Paraganglioma	Enrichment	SDHA, SDHB, SDHD	10.83
6	Pheochromocytoma/paraganglioma syndrome 1	Enrichment	SDHA, SDHB, SDHD	10.26
7	Paraganglioma and gastric stromal sarcoma	Enrichment	SDHB, SDHC, SDHD	9.91
8	Gastrointestinal stromal tumor	Enrichment	SDHA, SDHB, SDHC	9.43
9	Mitochondrial complex ii deficiency, nuclear type 1	Enrichment	SDHA, SDHAF1, SDHD	9.24
10	Cowden syndrome	Enrichment	SDHB, SDHC, SDHD	9.08
11	Pheochromocytoma	Enrichment	SDHA, SDHB, SDHD	8.45
12	Inherited cancer-predisposing syndrome	Enrichment	SDHA, SDHAF2, SDHB, SDHC, SDHD	8.35
13	Pheochromocytoma/paraganglioma syndrome 4	Enrichment	SDHB, SDHD	6.71
14	Sporadic pheochromocytoma/secreting paraganglioma	Enrichment	SDHB, SDHD	6.01
15	Multiple mitochondrial dysfunctions syndrome	Enrichment	ISCA1, ISCA2	5.69
16	Combined oxidative phosphorylation deficiency 19	Enrichment	LYRM4, NFS1	5.21
17	Rhabdomyosarcoma	Enrichment	SDHA, SDHC	5.11
18	Metaphyseal enchondromatosis with d-2-hydroxyglutaric aciduria	Enrichment	IDH1	3.83
19	Pheochromocytoma/paraganglioma syndrome 3	Enrichment	SDHC	3.53
20	Pheochromocytoma/paraganglioma syndrome 5	Enrichment	SDHA	3.53
21	Cardiomyopathy, dilated, 1gg	Enrichment	SDHA	3.53
22	Neurodegeneration with ataxia and late-onset optic atrophy	Enrichment	SDHA	3.53
23	Carcinoid syndrome	Enrichment	SDHD	3.53
24	Enchondromatosis	Enrichment	IDH1	3.35
25	Polymyoclonus, infantile	Enrichment	SDHA	3.23
26	Pheochromocytoma/paraganglioma syndrome 2	Enrichment	SDHAF2	3.23
27	Enchondromatosis, multiple, ollier type	Enrichment	IDH1	3.13
28	Paroxysmal extreme pain disorder	Enrichment	IDH1	3.13
29	Mitochondrial complex iv deficiency, nuclear type 2	Enrichment	SDHB	3.05
30	Mitochondrial complex iii deficiency, nuclear type 2	Enrichment	SDHD	3.05
31	Brain cancer	Enrichment	SDHA	3.05
32	Renal cell carcinoma	Enrichment	SDHB	3.05
33	Multiple enchondromatosis, maffucci type	Enrichment	IDH1	2.99
34	Pilocytic astrocytoma	Enrichment	SDHA	2.93
35	Glioma susceptibility 1	Enrichment	IDH1	2.93
36	Hereditary leiomyomatosis and renal cell cancer	Enrichment	FH	2.93
37	Oxoglutarate dehydrogenase deficiency	Enrichment	OGDH	2.93
38	Phosphoenolpyruvate carboxykinase deficiency, cytosolic	Enrichment	PCK1	2.93
39	Fumarase deficiency	Enrichment	FH	2.93
40	Retinitis pigmentosa 90	Enrichment	IDH3A	2.93
41	Pheochromocytoma/paraganglioma syndrome 7	Enrichment	DLST	2.93
42	Leiomyoma cutis	Enrichment	FH	2.93
43	Fumarate hydratase deficiency	Enrichment	FH	2.93
44	Mitochondrial dna depletion syndrome 9	Enrichment	SUCLG1	2.90
45	Retinitis pigmentosa 99	Enrichment	IDH3G	2.90
46	Mitochondrial dna depletion syndrome 5	Enrichment	SUCLA2	2.90
47	Retinitis pigmentosa 46	Enrichment	IDH3B	2.90
48	Developmental and epileptic encephalopathy 51	Enrichment	MDH2	2.90
49	Suclg1-related mitochondrial dna depletion syndrome, encephalomyopathic form with methylmalonic aciduria	Enrichment	SUCLG1	2.90
50	Gemistocytic astrocytoma	Enrichment	IDH2	2.90
51	Protoplasmic astrocytoma	Enrichment	IDH2	2.90
52	D-2-hydroxyglutaric aciduria 2	Enrichment	IDH2	2.90
53	Mixed oligodendroglioma-astrocytoma	Enrichment	IDH2	2.90
54	Anaplastic oligoastrocytoma	Enrichment	IDH2	2.90
55	Fibrillary astrocytoma	Enrichment	IDH2	2.90
56	Acute myocardial infarction	Enrichment	IDH2	2.90
57	Von hippel-lindau syndrome	Enrichment	SDHB	2.83
58	Multiple mitochondrial dysfunctions syndrome 4	Enrichment	ISCA2	2.83
59	Myopathy with lactic acidosis, hereditary	Enrichment	ISCU	2.83
60	Combined oxidative phosphorylation deficiency 52	Enrichment	NFS1	2.83
61	Mitochondrial complex ii deficiency, nuclear type 2	Enrichment	SDHAF1	2.75
62	Optic atrophy plus syndrome	Enrichment	ACO2, ISCA2	2.73
63	Leiomyoma, uterine	Enrichment	FH	2.63
64	Dihydrolipoamide dehydrogenase deficiency	Enrichment	DLD	2.63
65	Methemoglobinemia due to deficiency of methemoglobin reductase	Enrichment	DLD	2.63
66	Pyruvate dehydrogenase e2 deficiency	Enrichment	DLAT	2.63
67	Developmental and epileptic encephalopathy 88	Enrichment	MDH1	2.63
68	Spinocerebellar ataxia 45	Enrichment	FH	2.63
69	Gliosarcoma	Enrichment	IDH1	2.63
70	Porphyria, acute intermittent	Enrichment	ACO2	2.60
71	Acute porphyria	Enrichment	ACO2	2.60
72	Oligodendroglioma	Enrichment	IDH2	2.60
73	Anaplastic oligodendroglioma	Enrichment	IDH2	2.60
74	D-2-hydroxyglutaric aciduria	Enrichment	IDH2	2.60
75	Giant cell glioblastoma	Enrichment	IDH1	2.60
76	Glucocorticoid deficiency 4 with or without mineralocorticoid deficiency	Enrichment	NNT	2.60
77	Alpha-methylacetoacetic aciduria	Enrichment	ACAT1	2.53
78	Multiple mitochondrial dysfunctions syndrome 5	Enrichment	ISCA1	2.53
79	Pyruvate carboxylase deficiency	Enrichment	PC	2.45
80	Phosphoenolpyruvate carboxykinase deficiency	Enrichment	PCK1	2.45
81	Infantile cerebellar-retinal degeneration	Enrichment	ACO2	2.43
82	Optic atrophy 9	Enrichment	ACO2	2.43
83	Anaplastic astrocytoma	Enrichment	IDH2	2.43
84	Friedreich ataxia	Enrichment	FXN	2.35
85	Pyruvate dehydrogenase e1-alpha deficiency	Enrichment	PDHA1	2.33
86	Autosomal recessive isolated optic atrophy	Enrichment	ACO2	2.30
87	Spastic quadriplegic cerebral palsy	Enrichment	ISCA2	2.23
88	Leukemia, acute myeloid	Enrichment	IDH1	2.23
89	Familial glucocorticoid deficiency	Enrichment	NNT	1.90
90	Hereditary breast carcinoma	Enrichment	SDHA	1.89
91	Lactic acidosis	Enrichment	DLD	1.85
92	Congenital anomalies of kidney and urinary tract 1	Enrichment	TRAP1	1.83
93	Familial isolated dilated cardiomyopathy	Enrichment	SDHA	1.81
94	Isolated macular dystrophy	Enrichment	ACO2	1.76
95	Sudden infant death syndrome	Enrichment	PDHA1	1.70
96	Leigh syndrome, nuclear	Enrichment	SDHA	1.69
97	Breast cancer	Enrichment	SDHA	1.66
98	Leigh disease	Enrichment	SDHA	1.65
99	Hepatocellular carcinoma	Enrichment	FH	1.59
100	Ovarian cancer	Enrichment	FH, SDHAF2	1.58
101	Retinitis pigmentosa	Enrichment	IDH3A, IDH3B	1.55
102	Developmental and epileptic encephalopathy 1	Enrichment	MDH2	1.51
103	Hereditary retinal dystrophy	Enrichment	ACO2, IDH3A	1.30
104	Fundus dystrophy	Enrichment	ACO2, IDH3A	1.30
105	West syndrome	Enrichment	MDH2	1.27
106	Cakut	Enrichment	TRAP1	1.09

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

Citric acid cycle (TCA cycle)

Pathway Network for Citric acid cycle (TCA cycle)

Pathway network for the Citric acid cycle (TCA cycle) SuperPath

Member Pathways for Citric acid cycle (TCA cycle)

Pathways in the Citric acid cycle (TCA cycle) SuperPath

Gene overlap in member pathways for Citric acid cycle (TCA cycle) SuperPath

Associated Genes for Citric acid cycle (TCA cycle)

Associated Disorders for Citric acid cycle (TCA cycle)

Disorders associated with Citric acid cycle (TCA cycle) SuperPath

STRING Interaction Network for Citric acid cycle (TCA cycle)

Sources for Citric acid cycle (TCA cycle)