(S)-3-hydroxybutanoate

362,296 routes · scope human · up to 3 steps · built 5 October 2026 · Reactions · API

144 compounds within 3 steps: 2 in 1, 16 in 2, 126 in 3

Three steps

nitric oxideReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. 2 nitrite + AH2 + 2 H(+) = 2 nitric oxide + A + 2 H2O
Catalysed by ADHFE1, D2HGDH, SUOXmitochondrial matrix
N(2)-acetyl-L-ornithineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetate and acetoacetate (cytosol to extracellular)
3. N2-Acetyl-L-ornithine amidohydrolase
Catalysed by ADHFE1, SLC16A4, SLC16A5, SLC16A6 and 3 moremitochondrial matrix, extracellular space, cytosol
(S)-lactateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
Catalysed by ADHFE1, LDHA, LDHB, LDHC and 2 moremitochondrial matrix, cytosol
3'-deoxy-3',4'-didehydro-CTPReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. CTP + AH2 + S-adenosyl-L-methionine = 3'-deoxy-3',4'-didehydro-CTP + 5'-deoxyadenosine + L-methionine + A + H2O + H(+)
Catalysed by ADHFE1, D2HGDH, RSAD2mitochondrial matrix
2-oxobutanoateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
Catalysed by ADHFE1, AGXT2mitochondrial matrix, cytosolOne of 2 routes
2-oxoglutaramateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. L-Glutamine:pyruvate aminotransferase
Catalysed by ADHFE1, KYAT1mitochondrial matrix, cytosolOne of 2 routes
(S)-2-hydroxyglutarateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. (S)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
Catalysed by ADHFE1, D2HGDH, L2HGDHmitochondrial matrix
3-fumarylpyruvateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
Catalysed by ADHFE1, FAHD1mitochondrial matrix, cytosol
ferrocytochrome cReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and L-lactate (cytosol to extracellular)
3. (S)-Lactate:ferricytochrome-c 2-oxidoreductase
Catalysed by ADHFE1, SLC16A4, SLC16A5, SLC16A7 and 3 moremitochondrial matrix, extracellular space, cytosol
4-maleylacetoacetateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)
3. 4-maleylacetoacetate = 4-fumarylacetoacetate
Catalysed by ADHFE1, FAH, GSTZ1mitochondrial matrix
3-hydroxypyruvateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. L-serine + pyruvate = 3-hydroxypyruvate + L-alanine
Catalysed by ADHFE1, AGXTmitochondrial matrix, cytosol
17alpha-hydroxyprogesteroneReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetate and acetoacetate (cytosol to extracellular)
3. cytochrome P450 (17alpha-hydroxyprogesterone)
Catalysed by ADHFE1, SLC16A4, SLC16A5, SLC16A6 and 3 moremitochondrial matrix, extracellular space, cytosol, endoplasmic reticulum
aceneuramateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate
Catalysed by ADHFE1, NPLmitochondrial matrix, cytosol
5'-deoxyadenosineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O
Catalysed by ADHFE1, TYW1B, TYW1mitochondrial matrix, cytosolOne of 5 routes
3-hydroxy-3-methylglutarateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
3. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
Catalysed by ADHFE1, HMGCL, HMGCLL1, SUGCTmitochondrial matrixOne of 3 routes
3,3',5'-triiodo-L-thyronine sulfateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. 3,3',5'-triiodo-L-thyronine sulfate + iodide + A + H(+) = L-thyroxine sulfate + AH2
Catalysed by ADHFE1, D2HGDH, DIO1mitochondrial matrix
3,3'-diiodo-L-thyronineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. 3,3'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine + AH2
Catalysed by ADHFE1, D2HGDH, DIO1, DIO3mitochondrial matrixOne of 2 routes
3,3'-diiodo-L-thyronine sulfateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. 3,3'-diiodo-L-thyronine sulfate + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine sulfate + AH2
Catalysed by ADHFE1, D2HGDH, DIO1mitochondrial matrixOne of 2 routes
4-methylumbelliferyl acetateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetate and acetoacetate (cytosol to extracellular)
3. 4-methylumbelliferyl acetate + H2O = 4-methylumbelliferone + acetate + H(+)
Catalysed by ADHFE1, SLC16A4, SLC16A5, SLC16A6 and 5 moremitochondrial matrix, extracellular space, cytosol
urateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and L-lactate (cytosol to extracellular)
3. transport of L-lactate and urate (cytosol to extracellular)
Catalysed by ADHFE1, SLC16A4, SLC16A5, SLC16A7 and 3 moremitochondrial matrix, extracellular space, cytosol
all-trans-retinalReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and retinoate (cytosol to extracellular)
3. retinoate reduction
Catalysed by ADHFE1, SLC16A4, SLC16A5mitochondrial matrix, extracellular space, cytosol
fumaric acidReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)
3. 4-fumarylacetoacetate fumarylhydrolase
Catalysed by ADHFE1, FAHmitochondrial matrix, cytosolOne of 2 routes
dTDPReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. pyruvate kinase (dTDP)
Catalysed by ADHFE1, PKM, PKLRmitochondrial matrix, cytosol
3',5'-diiodo-L-thyronineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. 3',5'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5'-triiodo-L-thyronine + AH2
Catalysed by ADHFE1, D2HGDH, DIO3mitochondrial matrix
5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
Catalysed by ADHFE1, AGXT2mitochondrial matrix, cytosol
5-(3,3'-dimethylguanidino)-2-oxopentanoateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
Catalysed by ADHFE1, AGXT2mitochondrial matrix, cytosol
5-(3-methylguanidino)-2-oxopentanoateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. N(omega)-methyl-L-arginine + pyruvate = 5-(3-methylguanidino)-2-oxopentanoate + L-alanine
Catalysed by ADHFE1, AGXT2mitochondrial matrix, cytosol
3-iodothyronamineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. 3-iodothyronamine + iodide + A + H(+) = 3,5-diiodothyronamine + AH2
Catalysed by ADHFE1, D2HGDH, DIO1, DIO3mitochondrial matrixOne of 2 routes
3-iodo-L-thyronineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. 3-iodo-L-thyronine + iodide + A + H(+) = 3,5-diiodo-L-thyronine + AH2
Catalysed by ADHFE1, D2HGDH, DIO1, DIO3mitochondrial matrixOne of 2 routes
3'-iodo-L-thyronineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. 3'-iodo-L-thyronine + iodide + A + H(+) = 3',5'-diiodo-L-thyronine + AH2
Catalysed by ADHFE1, D2HGDH, DIO1, DIO2mitochondrial matrixOne of 2 routes
L-thyronineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. L-thyronine + iodide + A + H(+) = 3-iodo-L-thyronine + AH2
Catalysed by ADHFE1, D2HGDH, DIO3mitochondrial matrix
thyronamineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. thyronamine + iodide + A + H(+) = 3-iodothyronamine + AH2
Catalysed by ADHFE1, D2HGDH, DIO3mitochondrial matrix
3'-iodothyronamineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. 3'-iodothyronamine + iodide + A + H(+) = 3',5'-diiodothyronamine + AH2
Catalysed by ADHFE1, D2HGDH, DIO1, DIO2mitochondrial matrixOne of 2 routes
3,3'-diiodothyronamineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. 3,3'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2
Catalysed by ADHFE1, D2HGDH, DIO1, DIO2mitochondrial matrixOne of 2 routes
3',5'-diiodothyronamineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. 3',5'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2
Catalysed by ADHFE1, D2HGDH, DIO3mitochondrial matrix
3,3',5'-triiodothyronamineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. 3,3',5'-triiodothyronamine + iodide + A + H(+) = 3,3',5,5'-tetraiodothyronamine + AH2
Catalysed by ADHFE1, D2HGDH, DIO3mitochondrial matrix
hydroxyacetoneReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. Acetoacetate carboxy-lyase (acetone-forming)
3. cytochrome P450 (acetone)
Catalysed by ADHFE1, CYP2E1mitochondrial matrix, cytosol
17alpha-hydroxypregnenoloneReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetate and acetoacetate (cytosol to extracellular)
3. cytochrome P450 (17alpha-hydroxypregnenolone)
Catalysed by ADHFE1, SLC16A4, SLC16A5, SLC16A6 and 3 moremitochondrial matrix, extracellular space, cytosol, endoplasmic reticulum
chlorine(.)Reach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and formate (cytosol to extracellular)
3. transport of chloride and formate (cytosol to extracellular)
Catalysed by ADHFE1, SLC16A4, SLC16A5, SLC26A4mitochondrial matrix, extracellular space, cytosolOne of 2 routes
hydrogenselenideReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
Catalysed by ADHFE1, D2HGDH, SCLYmitochondrial matrix
3-hydroxypyruvic acidReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. L-Serine:pyruvate aminotransferase
Catalysed by ADHFE1, AGXTmitochondrial matrix, cytosolOne of 2 routes
3-oxopropanoateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
Catalysed by ADHFE1, AGXT2mitochondrial matrix, cytosol
D-serineReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. D-serine = pyruvate + NH4(+)
Catalysed by ADHFE1, SRRmitochondrial matrix, cytosol
(R)-mevalonateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
3. (R)-mevalonate + 2 NADP(+) + CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + 2 NADPH + 2 H(+)
Catalysed by ADHFE1, HMGCL, HMGCLL1, HMGCRmitochondrial matrix
(R)-lactic acidReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. (R)-Lactate:NAD+ oxidoreductase
Catalysed by ADHFE1, LDHDmitochondrial matrix, cytosol
25-hydroxycholesterolReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. (R)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2
3. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O
Catalysed by ADHFE1, D2HGDH, CH25Hmitochondrial matrix
2-iminopropanoateReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. 2-iminopropanoate + H2O = pyruvate + NH4(+)
Catalysed by ADHFE1, RIDAmitochondrial matrix, cytosol
phosphoenolpyruvic acidReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. pyruvate kinase (dTDP)
Catalysed by ADHFE1, PKM, PKLRmitochondrial matrix, cytosol
13-cis-retinalReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and retinoate (cytosol to extracellular)
3. retinal dehydrogenase (13-cis-retinal)
Catalysed by ADHFE1, SLC16A4, SLC16A5, ALDH1A2 and 1 moremitochondrial matrix, extracellular space, cytosol, endoplasmic reticulum
N-acetyl-beta-neuraminic acidReach from here →
1. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
Catalysed by ADHFE1, NPLmitochondrial matrix, cytosol