Ketone body metabolism

Pathway network for the Ketone body metabolism SuperPath

Sources:

Reactome
WikiPathways
PubChem

Pathways in the Ketone body metabolism SuperPath

#	Name	Source	Genes
1	Ketone body metabolism	Reactome	AACS ACAT1 ACSS3 BDH1 BDH2 HMGCL HMGCLL1 HMGCS2 OXCT1 OXCT2 (see all 10) (see less)
2	Synthesis of Ketone Bodies	Reactome	AACS ACAT1 ACSS3 BDH1 BDH2 HMGCL HMGCLL1 HMGCS2 (see all 8) (see less)
3	Ketogenesis and ketolysis	WikiPathways	ACAT1 BDH1 CPT2 OXCT1 SLC16A1 SLC25A20 SLC2A1 UCP2 (see all 8) (see less)
4	ketogenesis	PubChem	ACAT1 ACAT2 BDH1 HMGCL HMGCS1 HMGCS2 (see all 6) (see less)
5	Ketone bodies synthesis and degradation	WikiPathways	ACAT1 BDH1 HMGCL HMGCS2 OXCT1
6	Disorders in ketolysis	WikiPathways	ACAT1 ACAT2 BDH1 HMGCS1 OXCT1
7	ketolysis	PubChem	ACAT1 BDH1 BDH2 OXCT1
8	Disorders in ketone body synthesis	WikiPathways	ACAT1 BDH1 HMGCS2 OXCT1
9	Utilization of Ketone Bodies	Reactome	ACAT1 BDH1 OXCT1 OXCT2
10	glutaryl-CoA degradation	PubChem	ACAT1 ACAT2
11	Beta-ketothiolase deficiency	Reactome	ACAT1

Gene overlap in member pathways for Ketone body metabolism SuperPath

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

#	Symbol	Description	Category	# Related Pathways
1	ACAT1	Acetyl-CoA Acetyltransferase 1	Protein Coding	11
2	BDH1	3-Hydroxybutyrate Dehydrogenase 1	Protein Coding	9
3	OXCT1	3-Oxoacid CoA-Transferase 1	Protein Coding	7
4	HMGCS2	3-Hydroxy-3-Methylglutaryl-CoA Synthase 2	Protein Coding	5
5	HMGCL	3-Hydroxy-3-Methylglutaryl-CoA Lyase	Protein Coding	4
6	BDH2	3-Hydroxybutyrate Dehydrogenase 2	Protein Coding	3
7	ACAT2	Acetyl-CoA Acetyltransferase 2	Protein Coding	3
8	HMGCLL1	3-Hydroxy-3-Methylglutaryl-CoA Lyase Like 1	Protein Coding	2
9	OXCT2	3-Oxoacid CoA-Transferase 2	Protein Coding	2
10	AACS	Acetoacetyl-CoA Synthetase	Protein Coding	2
11	ACSS3	Acyl-CoA Synthetase Short Chain Family Member 3	Protein Coding	2
12	HMGCS1	3-Hydroxy-3-Methylglutaryl-CoA Synthase 1	Protein Coding	2
13	UCP2	Uncoupling Protein 2	Protein Coding	1
14	SLC25A20	Solute Carrier Family 25 Member 20	Protein Coding	1
15	CPT2	Carnitine Palmitoyltransferase 2	Protein Coding	1
16	SLC16A1	Solute Carrier Family 16 Member 1	Protein Coding	1
17	SLC2A1	Solute Carrier Family 2 Member 1	Protein Coding	1

Disorders associated with Ketone body metabolism SuperPath

according to GeneCards Suite gene sharing

#	Disorder	Type	Genes	Score
1	Alpha-methylacetoacetic aciduria	Direct
2	Amino acid metabolic disorder	Direct
3	Acetyl-coa acetyltransferase-2 deficiency	Enrichment	ACAT2	3.83
4	Succinyl-coa:3-oxoacid-coa transferase deficiency	Enrichment	OXCT1	3.53
5	3-hydroxy-3-methylglutaryl-coa lyase deficiency	Enrichment	HMGCL	3.43
6	3-hydroxy-3-methylglutaryl-coa synthase-2 deficiency	Enrichment	HMGCS2	3.23
7	Carnitine-acylcarnitine translocase deficiency	Enrichment	SLC25A20	3.23
8	Stomatin-deficient cryohydrocytosis with neurologic defects	Enrichment	SLC2A1	3.23
9	Erythrocyte lactate transporter defect	Enrichment	SLC16A1	3.23
10	Body mass index quantitative trait locus 4	Enrichment	UCP2	3.23
11	Epilepsy, idiopathic generalized 12	Enrichment	SLC2A1	3.23
12	Epilepsy with myoclonic absences	Enrichment	SLC2A1	3.23
13	Hyperinsulinism due to ucp2 deficiency	Enrichment	UCP2	3.23
14	Hereditary cryohydrocytosis with reduced stomatin	Enrichment	SLC2A1	3.23
15	Long-chain 3-hydroxyacyl-coa dehydrogenase deficiency	Enrichment	HMGCL	2.96
16	Dystonia 9	Enrichment	SLC2A1	2.93
17	Carnitine palmitoyltransferase ii deficiency, infantile	Enrichment	CPT2	2.93
18	Carnitine palmitoyltransferase ii deficiency, lethal neonatal	Enrichment	CPT2	2.93
19	Glut1 deficiency syndrome 1	Enrichment	SLC2A1	2.93
20	Monocarboxylate transporter 1 deficiency	Enrichment	SLC16A1	2.93
21	Carnitine palmitoyltransferase ii deficiency, myopathic, stress-induced	Enrichment	CPT2	2.93
22	Encephalopathy, acute, infection-induced 4	Enrichment	CPT2	2.93
23	Glucose transporter type 1 deficiency syndrome	Enrichment	SLC2A1	2.93
24	Hyperinsulinemic hypoglycemia, familial, 7	Enrichment	SLC16A1	2.93
25	Ketoacidosis due to monocarboxylate transporter-1 deficiency	Enrichment	SLC16A1	2.93
26	Acute necrotizing encephalopathy of childhood	Enrichment	CPT2	2.93
27	Glut1 deficiency syndrome 2	Enrichment	SLC2A1	2.75
28	Chromosome 17q23.1-q23.2 deletion syndrome	Enrichment	SLC2A1	2.75
29	Congenital myopathy 3 with rigid spine	Enrichment	HMGCS1	2.73
30	Paroxysmal dystonia	Enrichment	SLC2A1	2.38
31	Alternating hemiplegia of childhood	Enrichment	SLC2A1	2.33
32	Myoclonic-atonic epilepsy	Enrichment	SLC2A1	2.28
33	Developmental and epileptic encephalopathy 1	Enrichment	SLC2A1	1.83
34	Strabismus	Enrichment	SLC2A1	1.80
35	Epilepsy	Enrichment	SLC2A1	1.63
36	Benign epilepsy with centrotemporal spikes	Enrichment	SLC2A1	1.62
37	Centralopathic epilepsy	Enrichment	SLC2A1	1.60
38	West syndrome	Enrichment	SLC2A1	1.59
39	Body mass index quantitative trait locus 11	Enrichment	UCP2	1.54
40	Congenital nervous system abnormality	Enrichment	CPT2	1.22
41	Nervous system disease	Enrichment	CPT2	1.22
42	Microcephaly	Enrichment	SLC2A1	1.15

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

Ketone body metabolism

Pathway Network for Ketone body metabolism

Pathway network for the Ketone body metabolism SuperPath

Member Pathways for Ketone body metabolism

Pathways in the Ketone body metabolism SuperPath

Gene overlap in member pathways for Ketone body metabolism SuperPath

Associated Genes for Ketone body metabolism

Associated Disorders for Ketone body metabolism

Disorders associated with Ketone body metabolism SuperPath

STRING Interaction Network for Ketone body metabolism

Sources for Ketone body metabolism