Leucine, isoleucine and valine metabolism

Pathway network for the Leucine, isoleucine and valine metabolism SuperPath

Sources:

WikiPathways
Reactome
PubChem

Pathways in the Leucine, isoleucine and valine metabolism SuperPath

#	Name	Source	Genes
1	Leucine, isoleucine and valine metabolism	WikiPathways	ACAD8 ACADSB ACAT1 ACSF3 ALDH6A1 AUH BCAT1 BCAT2 BCKDHA BCKDHB DBT DLD ECHS1 HIBADH HIBCH HMGCLL1 HSD17B10 IVD MCCC1 MCCC2 MLYCD MMUT PCCA PCCB (see all 24) (see less)
2	Branched-chain amino acid catabolism	Reactome	ACAD8 ACADSB ACAT1 ALDH6A1 AUH BCAT1 BCAT2 BCKDHA BCKDHB BCKDK DBT DLD ECHS1 HIBADH HIBCH HSD17B10 IVD MCCC1 MCCC2 PPM1K SLC25A44 (see all 21) (see less)
3	valine degradation	PubChem	ABAT ACAD8 ALDH6A1 BCAT1 BCAT2 BCKDHA BCKDHB DBT DLD ECHS1 HADHA HIBADH HIBCH (see all 13) (see less)
4	Diseases of branched-chain amino acid catabolism	Reactome	ACAT1 AUH BCKDHA BCKDHB BCKDK DBT DLD ECHS1 HIBCH IVD MCCC1 MCCC2 PPM1K (see all 13) (see less)
5	isoleucine degradation	PubChem	ACADSB ACAT1 ACAT2 BCAT1 BCAT2 BCKDHA BCKDHB DBT DLD ECHS1 HADHA HSD17B10 (see all 12) (see less)
6	leucine degradation	PubChem	AUH BCAT1 BCAT2 BCKDHA BCKDHB DBT DLD HMGCL HMGCLL1 IVD MCCC1 MCCC2 (see all 12) (see less)
7	L-leucine degradation I	PubChem	AUH BCKDHA BCKDHB DBT DLD HMGCL MCCC1 MCCC2 (see all 8) (see less)
8	L-isoleucine degradation I	PubChem	ACAT1 BCKDHA BCKDHB DBT DLD HSD17B10 (see all 6) (see less)
9	L-valine degradation I	PubChem	ABAT BCKDHA BCKDHB DBT DLD
10	Branched-chain ketoacid dehydrogenase kinase deficiency	Reactome	BCKDHA BCKDHB BCKDK DBT DLD
11	3-methylglutaconic aciduria	Reactome	AUH
12	3-hydroxyisobutyryl-CoA hydrolase deficiency	Reactome	HIBCH

Gene overlap in member pathways for Leucine, isoleucine and valine metabolism SuperPath

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

#	Symbol	Description	Category	# Related Pathways
1	BCKDHA	Branched Chain Keto Acid Dehydrogenase E1 Subunit Alpha	Protein Coding	10
2	DBT	Dihydrolipoamide Branched Chain Transacylase E2	Protein Coding	10
3	BCKDHB	Branched Chain Keto Acid Dehydrogenase E1 Subunit Beta	Protein Coding	10
4	DLD	Dihydrolipoamide Dehydrogenase	Protein Coding	10
5	AUH	AU RNA Binding Methylglutaconyl-CoA Hydratase	Protein Coding	6
6	BCAT1	Branched Chain Amino Acid Transaminase 1	Protein Coding	5
7	BCAT2	Branched Chain Amino Acid Transaminase 2	Protein Coding	5
8	HIBCH	3-Hydroxyisobutyryl-CoA Hydrolase	Protein Coding	5
9	MCCC1	Methylcrotonyl-CoA Carboxylase Subunit 1	Protein Coding	5
10	MCCC2	Methylcrotonyl-CoA Carboxylase Subunit 2	Protein Coding	5
11	ACAT1	Acetyl-CoA Acetyltransferase 1	Protein Coding	5
12	ECHS1	Enoyl-CoA Hydratase, Short Chain 1	Protein Coding	5
13	IVD	Isovaleryl-CoA Dehydrogenase	Protein Coding	4
14	HSD17B10	Hydroxysteroid 17-Beta Dehydrogenase 10	Protein Coding	4
15	ACAD8	Acyl-CoA Dehydrogenase Family Member 8	Protein Coding	3
16	ALDH6A1	Aldehyde Dehydrogenase 6 Family Member A1	Protein Coding	3
17	HIBADH	3-Hydroxyisobutyrate Dehydrogenase	Protein Coding	3
18	ACADSB	Acyl-CoA Dehydrogenase Short/Branched Chain	Protein Coding	3
19	BCKDK	Branched Chain Keto Acid Dehydrogenase Kinase	Protein Coding	3
20	HMGCLL1	3-Hydroxy-3-Methylglutaryl-CoA Lyase Like 1	Protein Coding	2
21	PPM1K	Protein Phosphatase, Mg2+/Mn2+ Dependent 1K	Protein Coding	2
22	HADHA	Hydroxyacyl-CoA Dehydrogenase Trifunctional Multienzyme Complex Subunit Alpha	Protein Coding	2
23	ABAT	4-Aminobutyrate Aminotransferase	Protein Coding	2
24	HMGCL	3-Hydroxy-3-Methylglutaryl-CoA Lyase	Protein Coding	2
25	MMUT	Methylmalonyl-CoA Mutase	Protein Coding	1
26	PCCA	Propionyl-CoA Carboxylase Subunit Alpha	Protein Coding	1
27	PCCB	Propionyl-CoA Carboxylase Subunit Beta	Protein Coding	1
28	ACSF3	Acyl-CoA Synthetase Family Member 3	Protein Coding	1
29	MLYCD	Malonyl-CoA Decarboxylase	Protein Coding	1
30	SLC25A44	Solute Carrier Family 25 Member 44	Protein Coding	1
31	ACAT2	Acetyl-CoA Acetyltransferase 2	Protein Coding	1

Disorders associated with Leucine, isoleucine and valine metabolism SuperPath

according to GeneCards Suite gene sharing

#	Disorder	Type	Genes	Score
1	Amino acid metabolic disorder	Direct
2	Branched-chain keto acid dehydrogenase kinase deficiency	Direct
3	3-methylglutaconic aciduria	Direct
4	3-hydroxyisobutryl-coa hydrolase deficiency	Direct
5	Maple syrup urine disease, type ia	Enrichment	BCKDHA, BCKDHB, DBT	10.73
6	Intermittent maple syrup urine disease	Enrichment	BCKDHA, BCKDHB, DBT	10.73
7	Classic maple syrup urine disease	Enrichment	BCKDHA, BCKDHB, DBT	10.73
8	Intermediate maple syrup urine disease	Enrichment	BCKDHA, BCKDHB, DBT, PPM1K	10.43
9	Dihydrolipoamide dehydrogenase deficiency	Enrichment	BCKDHB, DLD	6.96
10	3-methylcrotonyl-coa carboxylase deficiency	Enrichment	MCCC1, MCCC2	6.52
11	Alpha-methylacetoacetic aciduria	Enrichment	ACAT1, ACAT2	6.14
12	Propionic acidemia	Enrichment	PCCA, PCCB	4.75
13	Isolated methylmalonic acidemia	Enrichment	ACSF3, MMUT	4.20
14	Methylmalonic acidemia	Enrichment	ACSF3, MMUT	4.08
15	Maple syrup urine disease, type ii	Enrichment	DBT	3.43
16	Gaba aminotransferase deficiency	Enrichment	ABAT	3.43
17	Gaba-transaminase deficiency	Enrichment	ABAT	3.43
18	Maple syrup urine disease, type ib	Enrichment	BCKDHB	3.43
19	Hsd10 mitochondrial disease	Enrichment	HSD17B10	3.35
20	Syndromic x-linked intellectual disability type 10	Enrichment	HSD17B10	3.35
21	3-hydroxy-3-methylglutaryl-coa lyase deficiency	Enrichment	HMGCL	3.23
22	3-methylcrotonyl-coa carboxylase 2 deficiency	Enrichment	MCCC2	3.23
23	Methemoglobinemia due to deficiency of methemoglobin reductase	Enrichment	DLD	3.13
24	2-methylbutyryl-coa dehydrogenase deficiency	Enrichment	ACADSB	3.05
25	Hypervalinemia and hyperleucine-isoleucinemia	Enrichment	BCAT2	3.05
26	Acetyl-coa acetyltransferase-2 deficiency	Enrichment	ACAT2	3.05
27	Acute fatty liver of pregnancy	Enrichment	HADHA	3.05
28	Isovaleric acidemia	Enrichment	IVD	3.05
29	Isobutyryl-coa dehydrogenase deficiency	Enrichment	ACAD8	3.02
30	3-hydroxyisobutyryl-coa hydrolase deficiency	Enrichment	HIBCH	3.02
31	3-methylcrotonyl-coa carboxylase 1 deficiency	Enrichment	MCCC1	2.93
32	Maple syrup urine disease, mild variant	Enrichment	PPM1K	2.81
33	3-methylglutaconic aciduria, type i	Enrichment	AUH	2.75
34	Spinal muscular atrophy, type iv	Enrichment	MCCC2	2.75
35	Long-chain 3-hydroxyacyl-coa dehydrogenase deficiency	Enrichment	HMGCL	2.75
36	Mitochondrial short-chain enoyl-coa hydratase 1 deficiency	Enrichment	ECHS1	2.75
37	Methylmalonate semialdehyde dehydrogenase deficiency	Enrichment	ALDH6A1	2.72
38	Mitochondrial trifunctional protein deficiency 1	Enrichment	HADHA	2.58
39	Mitochondrial trifunctional protein deficiency	Enrichment	HADHA	2.58
40	Combined malonic and methylmalonic aciduria	Enrichment	ACSF3	2.45
41	Lactic acidosis	Enrichment	DLD	2.35
42	Methylmalonic aciduria due to methylmalonyl-coa mutase deficiency	Enrichment	MMUT	2.28
43	Malonyl-coa decarboxylase deficiency	Enrichment	MLYCD	2.15
44	Liver failure, infantile, transient	Enrichment	MMUT	2.05
45	Pontocerebellar hypoplasia, type 2d	Enrichment	PCCA	2.05
46	Leigh syndrome, nuclear	Enrichment	ECHS1	1.23
47	Autism spectrum disorder	Enrichment	MCCC2	1.20
48	Leigh disease	Enrichment	ECHS1	1.19

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

Leucine, isoleucine and valine metabolism

Pathway Network for Leucine, isoleucine and valine metabolism

Pathway network for the Leucine, isoleucine and valine metabolism SuperPath

Member Pathways for Leucine, isoleucine and valine metabolism

Pathways in the Leucine, isoleucine and valine metabolism SuperPath

Gene overlap in member pathways for Leucine, isoleucine and valine metabolism SuperPath

Associated Genes for Leucine, isoleucine and valine metabolism

Associated Disorders for Leucine, isoleucine and valine metabolism

Disorders associated with Leucine, isoleucine and valine metabolism SuperPath

STRING Interaction Network for Leucine, isoleucine and valine metabolism

Sources for Leucine, isoleucine and valine metabolism