Mitochondrial Fatty Acid Beta-Oxidation

Pathway network for the Mitochondrial Fatty Acid Beta-Oxidation SuperPath

Sources:

Reactome

Pathways in the Mitochondrial Fatty Acid Beta-Oxidation SuperPath

#	Name	Source	Genes
1	Mitochondrial Fatty Acid Beta-Oxidation	Reactome	ACAA2 ACAD10 ACAD11 ACADL ACADM ACADS ACADVL ACBD6 ACBD7 ACOT1 ACOT11 ACOT12 ACOT13 ACOT2 ACOT7 ACOT9 ACSF2 ACSM3 ACSM6 DBI DECR1 ECHS1 ECI1 HADH HADHA HADHB MCAT MCEE MECR MMAA MMUT NDUFAB1 PCCA PCCB PCTP THEM4 THEM5 (see all 37) (see less)
2	mitochondrial fatty acid beta-oxidation of saturated fatty acids	Reactome	ACADL ACADM ACADS ACADVL ACSM3 ACSM6 ECHS1 HADH HADHA HADHB MECR (see all 11) (see less)
3	Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA	Reactome	ACADM ECHS1 HADH HADHA HADHB MECR (see all 6) (see less)
4	Beta oxidation of hexanoyl-CoA to butanoyl-CoA	Reactome	ACADS ECHS1 HADH HADHA HADHB
5	Beta oxidation of octanoyl-CoA to hexanoyl-CoA	Reactome	ACADM ECHS1 HADH HADHA HADHB
6	Propionyl-CoA catabolism	Reactome	MCEE MMAA MMUT PCCA PCCB
7	Beta oxidation of butanoyl-CoA to acetyl-CoA	Reactome	ACADS ACSM3 ACSM6 ECHS1 HADH
8	Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA	Reactome	ACADL ECHS1 HADH HADHA HADHB
9	Beta oxidation of palmitoyl-CoA to myristoyl-CoA	Reactome	ACADVL HADHA HADHB
10	Beta oxidation of myristoyl-CoA to lauroyl-CoA	Reactome	ACADL HADHA HADHB
11	Mitochondrial short-chain enoyl-CoA hydratase deficiency 1	Reactome	ECHS1

Gene overlap in member pathways for Mitochondrial Fatty Acid Beta-Oxidation SuperPath

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

#	Symbol	Description	Category	# Related Pathways
1	ECHS1	Enoyl-CoA Hydratase, Short Chain 1	Protein Coding	8
2	HADHA	Hydroxyacyl-CoA Dehydrogenase Trifunctional Multienzyme Complex Subunit Alpha	Protein Coding	8
3	HADHB	Hydroxyacyl-CoA Dehydrogenase Trifunctional Multienzyme Complex Subunit Beta	Protein Coding	8
4	HADH	Hydroxyacyl-CoA Dehydrogenase	Protein Coding	7
5	ACADL	Acyl-CoA Dehydrogenase Long Chain	Protein Coding	4
6	ACADM	Acyl-CoA Dehydrogenase Medium Chain	Protein Coding	4
7	ACADS	Acyl-CoA Dehydrogenase Short Chain	Protein Coding	4
8	ACSM6	Acyl-CoA Synthetase Medium Chain Family Member 6	Protein Coding	3
9	ACADVL	Acyl-CoA Dehydrogenase Very Long Chain	Protein Coding	3
10	MECR	Mitochondrial Trans-2-Enoyl-CoA Reductase	Protein Coding	3
11	ACSM3	Acyl-CoA Synthetase Medium Chain Family Member 3	Protein Coding	3
12	MMAA	Metabolism Of Cobalamin Associated A	Protein Coding	2
13	MMUT	Methylmalonyl-CoA Mutase	Protein Coding	2
14	PCCA	Propionyl-CoA Carboxylase Subunit Alpha	Protein Coding	2
15	PCCB	Propionyl-CoA Carboxylase Subunit Beta	Protein Coding	2
16	MCEE	Methylmalonyl-CoA Epimerase	Protein Coding	2
17	ACAA2	Acetyl-CoA Acyltransferase 2	Protein Coding	1
18	ACOT2	Acyl-CoA Thioesterase 2	Protein Coding	1
19	ACOT7	Acyl-CoA Thioesterase 7	Protein Coding	1
20	THEM4	Thioesterase Superfamily Member 4	Protein Coding	1
21	ACOT12	Acyl-CoA Thioesterase 12	Protein Coding	1
22	DBI	Diazepam Binding Inhibitor, Acyl-CoA Binding Protein	Protein Coding	1
23	ECI1	Enoyl-CoA Delta Isomerase 1	Protein Coding	1
24	DECR1	2,4-Dienoyl-CoA Reductase 1	Protein Coding	1
25	ACOT9	Acyl-CoA Thioesterase 9	Protein Coding	1
26	ACOT11	Acyl-CoA Thioesterase 11	Protein Coding	1
27	MCAT	Malonyl-CoA-Acyl Carrier Protein Transacylase	Protein Coding	1
28	THEM5	Thioesterase Superfamily Member 5	Protein Coding	1
29	ACBD7	Acyl-CoA Binding Domain Containing 7	Protein Coding	1
30	NDUFAB1	NADH:Ubiquinone Oxidoreductase Subunit AB1	Protein Coding	1
31	ACOT13	Acyl-CoA Thioesterase 13	Protein Coding	1
32	PCTP	Phosphatidylcholine Transfer Protein	Protein Coding	1
33	ACOT1	Acyl-CoA Thioesterase 1	Protein Coding	1
34	ACSF2	Acyl-CoA Synthetase Family Member 2	Protein Coding	1
35	ACAD10	Acyl-CoA Dehydrogenase Family Member 10	Protein Coding	1
36	ACAD11	Acyl-CoA Dehydrogenase Family Member 11	Protein Coding	1
37	ACBD6	Acyl-CoA Binding Domain Containing 6	Protein Coding	1

Disorders associated with Mitochondrial Fatty Acid Beta-Oxidation SuperPath

according to GeneCards Suite gene sharing

#	Disorder	Type	Genes	Score
1	Mitochondrial short-chain enoyl-coa hydratase 1 deficiency	Direct
2	Isolated methylmalonic acidemia	Enrichment	MCEE, MMAA, MMUT	9.06
3	Methylmalonic acidemia	Enrichment	MCEE, MMAA, MMUT	8.86
4	Mitochondrial trifunctional protein deficiency 1	Enrichment	HADHA, HADHB	7.01
5	Mitochondrial trifunctional protein deficiency	Enrichment	HADHA, HADHB	7.01
6	Propionic acidemia	Enrichment	PCCA, PCCB	6.19
7	Mitochondrial trifunctional protein deficiency 2	Enrichment	HADHB	3.66
8	Acute fatty liver of pregnancy	Enrichment	HADHA	3.66
9	3-hydroxyacyl-coa dehydrogenase deficiency	Enrichment	HADH	3.43
10	Acyl-coa dehydrogenase, medium-chain, deficiency of	Enrichment	ACADM	3.43
11	Medium-chain acyl-coenzyme a dehydrogenase deficiency	Enrichment	ACADM	3.43
12	Methylmalonyl-coa epimerase deficiency	Enrichment	MCEE	3.43
13	Methylmalonic aciduria, cbla type	Enrichment	MMAA	3.43
14	Vitamin b12-responsive methylmalonic acidemia	Enrichment	MMAA	3.43
15	Optic atrophy 16	Enrichment	MECR	3.35
16	Dystonia, childhood-onset, with optic atrophy and basal ganglia abnormalities	Enrichment	MECR	3.35
17	Heart defects, congenital, and other congenital anomalies	Enrichment	ACADVL	3.18
18	Long-chain 3-hydroxyacyl-coa dehydrogenase deficiency	Enrichment	HADHA	3.18
19	Intellectual developmental disorder, autosomal dominant 62	Enrichment	ACADVL	3.18
20	Dlg4-related synaptopathy	Enrichment	ACADVL	3.18
21	Acyl-coa dehydrogenase, short-chain, deficiency of	Enrichment	ACADS	3.13
22	Hyperinsulinemic hypoglycemia, familial, 4	Enrichment	HADH	3.13
23	Neurodevelopmental disorder with speech delay and variable ocular anomalies	Enrichment	ACSM3	3.13
24	Acyl-coa dehydrogenase, very long-chain, deficiency of	Enrichment	ACADVL	3.05
25	Hyperinsulinemic hypoglycemia	Enrichment	HADH	2.96
26	Methylmalonic aciduria due to methylmalonyl-coa mutase deficiency	Enrichment	MMUT	2.96
27	Hydrocephalus, congenital, 2, with or without brain or eye anomalies	Enrichment	ACADM	2.83
28	Liver failure, infantile, transient	Enrichment	MMUT	2.73
29	Pontocerebellar hypoplasia, type 2d	Enrichment	PCCA	2.73
30	Hyperinsulinemic hypoglycemia, familial, 1	Enrichment	HADH	2.66
31	Nonsyndromic genetic hyperinsulinism	Enrichment	HADH	2.66
32	Optic atrophy 15	Enrichment	MCAT	2.56
33	Neurodevelopmental disorder with progressive movement abnormalities	Enrichment	ACBD6	2.56
34	Pituitary hormone deficiency, combined, 4	Enrichment	ACBD6	2.09
35	Myopathy	Enrichment	ACADVL	2.05
36	Autosomal recessive isolated optic atrophy	Enrichment	MCAT	1.96
37	Optic atrophy plus syndrome	Enrichment	MECR	1.71
38	Leigh syndrome, nuclear	Enrichment	ECHS1	1.60
39	Leigh disease	Enrichment	ECHS1	1.56
40	Mitochondrial disease	Enrichment	MECR	1.42

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 Fatty Acid Beta-Oxidation

Pathway Network for Mitochondrial Fatty Acid Beta-Oxidation

Pathway network for the Mitochondrial Fatty Acid Beta-Oxidation SuperPath

Member Pathways for Mitochondrial Fatty Acid Beta-Oxidation

Pathways in the Mitochondrial Fatty Acid Beta-Oxidation SuperPath

Gene overlap in member pathways for Mitochondrial Fatty Acid Beta-Oxidation SuperPath

Associated Genes for Mitochondrial Fatty Acid Beta-Oxidation

Associated Disorders for Mitochondrial Fatty Acid Beta-Oxidation

Disorders associated with Mitochondrial Fatty Acid Beta-Oxidation SuperPath

STRING Interaction Network for Mitochondrial Fatty Acid Beta-Oxidation

Sources for Mitochondrial Fatty Acid Beta-Oxidation