Valine, leucine and isoleucine biosynthesis

Source	ID	Pathway
KEGG	00290

Pathway Metabolites

YMDB ID	Name CAS Number	IUPAC Name	Formula Weight
YMDB00038	L-Isoleucine 73-32-5	(2S,3S)-2-amino-3-methylpentanoic acid	C₆H₁₃NO₂ 131.1729
YMDB00039	(S)-2-Aceto-2-hydroxybutanoic acid	(2S)-2-ethyl-2-hydroxy-3-oxobutanoic acid	C₆H₁₀O₄ 146.1412
YMDB00063	(R)-2,3-Dihydroxy-isovalerate	(2R)-2,3-dihydroxy-3-methylbutanoic acid	C₅H₁₀O₄ 134.1305
YMDB00068	(R)-2,3-Dihydroxy-3-methylvalerate 562-43-6	(2R,3R)-2,3-dihydroxy-3-methylpentanoic acid	C₆H₁₂O₄ 148.1571
YMDB00071	2-Ketobutyric acid 600-18-0	2-oxobutanoic acid	C₄H₆O₃ 102.0886
YMDB00106	2-Isopropylmalic acid 49601-06-1	(2S)-2-hydroxy-2-(propan-2-yl)butanedioic acid	C₇H₁₂O₅ 176.1672
YMDB00152	L-Valine 72-18-4	(2S)-2-amino-3-methylbutanoic acid	C₅H₁₁NO₂ 117.1463
YMDB00168	(S)-3-methyl-2-oxovaleric acid 1460-34-0	(3S)-3-methyl-2-oxopentanoic acid	C₆H₁₀O₃ 130.1418
YMDB00175	Pyruvic acid 127-17-3	2-oxopropanoic acid	C₃H₄O₃ 88.0621
YMDB00177	(S)-2-Acetolactate	(2S)-2-hydroxy-2-methyl-3-oxobutanoic acid	C₅H₈O₄ 132.1146
YMDB00214	L-Threonine 72-19-5	(2S,3R)-2-amino-3-hydroxybutanoic acid	C₄H₉NO₃ 119.1192
YMDB00312	Acetyl-CoA 72-89-9	{[(2R,3S,4R,5R)-2-({[({[(3R)-3-[(2-{[2-(acetylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]-3-hydroxy-2,2-dimethylpropoxy](hydroxy)phosphoryl}oxy)(hydroxy)phosphoryl]oxy}methyl)-5-(6-amino-9H-purin-9-yl)-4-hydroxyoxolan-3-yl]oxy}phosphonic acid	C₂₃H₃₈N₇O₁₇P₃S 809.571
YMDB00365	Alpha-Ketoisovaleric acid 759-05-7	3-methyl-2-oxobutanoic acid	C₅H₈O₃ 116.1152
YMDB00368	3-Isopropylmalate 921-28-8	(2R,3S)-2-hydroxy-3-(propan-2-yl)butanedioic acid	C₇H₁₂O₅ 176.1672
YMDB00387	L-Leucine 61-90-5	(2S)-2-amino-4-methylpentanoic acid	C₆H₁₃NO₂ 131.1729
YMDB00388	Ketoleucine 816-66-0	4-methyl-2-oxopentanoic acid	C₆H₁₀O₃ 130.1418
YMDB00428	(S)-2-acetyl-2-hydroxybutanoate	(2S)-2-ethyl-2-hydroxy-3-oxobutanoic acid	C₆H₁₀O₄ 146.1412
YMDB00442	2-Isopropylmaleic acid	(2Z)-2-(propan-2-yl)but-2-enedioic acid	C₇H₁₀O₄ 158.1519
YMDB01004	(2R,3S)-3-isopropylmalate(2-) 921-28-8		C₁₈H₃₅O₂ 283.4693
YMDB01011	2-oxobutanoate 600-18-0		C₄H₅O₃ 101.0807
YMDB01017	3-methyl-2-oxobutanoate 759-05-7		C₅H₇O₃ 115.1073
YMDB01020	(S)-3-methyl-2-oxovalerate 1460-34-0		C₆H₉O₃ 129.1339
YMDB01030	4-Methyl-2-oxopentanoate 816-66-0		C₆H₉O₃ 129.1339
YMDB01053	pyruvate 127-17-3		C₃H₃O₃ 87.0541
YMDB01070	(R)-2,3-Dihydroxy-3-methylbutanoate	(2R)-2,3-dihydroxy-3-methylbutanoate	C₅H₉O₄ 133.1226
YMDB01091	(2R,3R)-2,3-Dihydroxy-3-methylpentanoate	(2R,3R)-2,3-dihydroxy-3-methylpentanoate	C₆H₁₁O₄ 147.1491

Pathway Proteins

Uniprot ID	Gene Name Locus	Name	Type	Metabolites
P47176	BAT2 YJR148W	Branched-chain-amino-acid aminotransferase, cytosolic	Enzyme	L-Isoleucine L-Valine Oxoglutaric acid (S)-3-methyl-2-oxovaleric acid L-Glutamic acid Alpha-Ketoisovaleric acid L-Leucine Ketoleucine (2S)-2-Isopropyl-3-oxosuccinate hydron Carbon dioxide
P48526	ISM1 YPL040C	Isoleucyl-tRNA synthetase, mitochondrial	Enzyme	L-Isoleucine Adenosine monophosphate Adenosine triphosphate Pyrophosphate
P38891	BAT1 YHR208W	Branched-chain-amino-acid aminotransferase, mitochondrial	Enzyme	L-Isoleucine L-Valine Oxoglutaric acid (S)-3-methyl-2-oxovaleric acid L-Glutamic acid Alpha-Ketoisovaleric acid L-Leucine Ketoleucine (2S)-2-Isopropyl-3-oxosuccinate hydron Carbon dioxide
P09436	ILS1 YBL076C	Isoleucyl-tRNA synthetase, cytoplasmic	Enzyme	L-Isoleucine Adenosine monophosphate Adenosine triphosphate Pyrophosphate
P06168	ILV5 YLR355C	Ketol-acid reductoisomerase, mitochondrial	Enzyme	(S)-2-Aceto-2-hydroxybutanoic acid (R)-2,3-Dihydroxy-isovalerate (R)-2,3-Dihydroxy-3-methylvalerate 2-Dehydropantoic acid (S)-2-Acetolactate (R)-Pantoic acid 2-Acetolactate NADPH NADP (S)-2-acetyl-2-hydroxybutanoate hydron
Q12166	LEU9 YOR108W	2-isopropylmalate synthase 2, mitochondrial	Enzyme	Coenzyme A 2-Isopropylmalic acid Acetyl-CoA Alpha-Ketoisovaleric acid hydron water
P06208	LEU4 YNL104C	2-isopropylmalate synthase	Enzyme	Coenzyme A 2-Isopropylmalic acid Acetyl-CoA Alpha-Ketoisovaleric acid hydron water
P00927	ILV1 YER086W	Threonine dehydratase, mitochondrial	Enzyme	2-Ketobutyric acid Ammonia L-Threonine Ammonium
P25379	CHA1 YCL064C	Catabolic L-serine/threonine dehydratase	Enzyme	2-Ketobutyric acid Ammonia L-Serine Pyruvic acid L-Threonine Ammonium hydron water 2-aminoprop-2-enoate
P07806	VAS1 YGR094W	Valyl-tRNA synthetase, mitochondrial	Enzyme	Adenosine monophosphate Adenosine triphosphate L-Valine Pyrophosphate
P26637	CDC60 YPL160W	Leucyl-tRNA synthetase, cytoplasmic	Enzyme	Adenosine monophosphate Adenosine triphosphate Pyrophosphate L-Leucine
P11325	NAM2 YLR382C	Leucyl-tRNA synthetase, mitochondrial	Enzyme	Adenosine monophosphate Adenosine triphosphate Pyrophosphate L-Leucine
P07264	LEU1 YGL009C	3-isopropylmalate dehydratase	Enzyme	2-Isopropylmalic acid 3-Isopropylmalate 2-Isopropylmaleic acid water
P04173	LEU2 YCL018W	3-isopropylmalate dehydrogenase	Enzyme	NAD NADH 3-Isopropylmalate Ketoleucine NAD(+) (2S)-2-Isopropyl-3-oxosuccinate hydron Carbon dioxide
P16387	PDA1 YER178W	Pyruvate dehydrogenase E1 component subunit alpha, mitochondrial	Enzyme	Pyruvic acid (6R)-5,10-Methenyltetrahydrofolic acid 5-Formyltetrahydrofolic acid hydron water Carbon dioxide
P32473	PDB1 YBR221C	Pyruvate dehydrogenase E1 component subunit beta, mitochondrial	Enzyme	Coenzyme A NAD NADH Pyruvic acid Acetyl-CoA Thiamine pyrophosphate Carbon dioxide 2-(a-Hydroxyethyl)thiamine diphosphate
P07342	ILV2 YMR108W	Acetolactate synthase catalytic subunit, mitochondrial	Enzyme	2-Ketobutyric acid Pyruvic acid (S)-2-Acetolactate 2-Acetolactate (S)-2-acetyl-2-hydroxybutanoate hydron Carbon dioxide
P39522	ILV3 YJR016C	Dihydroxy-acid dehydratase, mitochondrial	Enzyme	(R)-2,3-Dihydroxy-isovalerate (R)-2,3-Dihydroxy-3-methylvalerate (S)-3-methyl-2-oxovaleric acid Alpha-Ketoisovaleric acid water 3-Methylbutanoic acid 2,3-Dihydroxyisovaleric acid