KMO
UniProt:O15229 KMO
PIG6
POX2
PRODH
UniProt:O43272 PRODH
TPO
UniProt:P07202 TPO
PENT
PNMT
UniProt:P11086 PNMT
ODC1
UniProt:P11926 ODC1
AMD
AMD1
UniProt:P17707 AMD1
SPS1
SRM
SRML1
UniProt:P19623 SRM
SAT
SAT1
UniProt:P21673 SAT1
CPS1
UniProt:P31327 CPS1
HAL
HIS
UniProt:P42357 HAL
ASMT
UniProt:P46597 ASMT
SLC6A8
UniProt:P48029 SLC6A8
SMS
UniProt:P52788 SMS
DIO3
ITDI3
TXDI3
UniProt:P55073 DIO3
ALDH6A1
MMSDH
UniProt:Q02252 ALDH6A1
GAMT
UniProt:Q14353 GAMT
AANAT
SNAT
UniProt:Q16613 AANAT
TST
UniProt:Q16762 TST
AFMID
UniProt:Q63HM1 AFMID
HIBCH
UniProt:Q6NVY1 HIBCH
NAGS
UniProt:Q8N159 NAGS
NIS
SLC5A5
UniProt:Q92911 SLC5A5
ADC
KIAA1945
ODCP
UniProt:Q96A70 ADC
AMDHD1
HMFT1272
UniProt:Q96NU7 AMDHD1
ODC
SLC25A21
UniProt:Q9BQT8 SLC25A21
AGMAT
UniProt:Q9BSE5 AGMAT
MRDI
MRI1
UniProt:Q9BV20 MRI1
C20orf16
SMO
SMOX
UniProt:Q9NWM0 SMOX
GLXR
GRHPR
MSTP035
UniProt:Q9UBQ7 GRHPR
DIC
SLC25A10
UniProt:Q9UBX3 SLC25A10
Sulfate is exported to the cytosol in exchange for dicarboxylate
phenylalanine + tetrahydrobiopterin + O2 => tyrosine + 4a-hydroxytetrahydrobiopterin + H2O
kynurenine + pyruvate => 4-(2-aminophenyl)-2,4-dioxobutanoic acid + alanine [CCBL1]
glutamine + H2O => glutamate + NH4+ [GLS]
glutamate + NH4+ + ATP => glutamine + ADP + orthophosphate [GLUL]
oxaloacetate + glutamate <=> aspartate + alpha-ketoglutarate [GOT2]
aspartate + alpha-ketoglutarate <=> oxaloacetate + glutamate [GOT1]
MTR transfers CH3 group from MeCbl to HCYS
Methionine is reformed
alanine + alpha-ketoglutarate <=> pyruvate + glutamate [GPT]
pyruvate + glutamate <=> alanine + alpha-ketoglutarate [GPT]
alanine + alpha-ketoglutarate <=> pyruvate + glutamate [GPT2]
pyruvate + glutamate <=> alanine + alpha-ketoglutarate [GPT2]
oxaloacetate + glutamate <=> aspartate + alpha-ketoglutarate [GOT1]
aspartate + alpha-ketoglutarate <=> oxaloacetate + glutamate [GOT2]
ornithine + alpha-ketoglutarate <=> glutamate + L-glutamate gamma-semialdehyde [OAT]
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT]
glutamate + ATP + NADPH + H+ => L-glutamate gamma-semialdehyde + NADP+ + ADP + orthophosphate [P5CS]
L-glutamate gamma-semialdehyde <=> L-1-pyrroline-5-carboxylate
L-1-pyrroline-5-carboxylate + NADPH + H+ => proline + NADP+
aspartate + glutamine + ATP <=> asparagine + glutamate + AMP + pyrophosphate [ASNS]
alpha-ketoglutarate + NH4+ + NAD(P)H + H+ <=> glutamate + NAD(P)+ [GLUD1]
glutamate + NAD(P)+ => alpha-ketoglutarate + NH4+ + NAD(P)H + H+ [GLUD1]
glutamine + pyruvate => 2-oxoglutaramate + alanine
3-phosphoglycerate is dehydrogenated
3-phosphonooxypyruvic acid + L-glutamate = O-phospho-L-serine + 2-oxoglutarate
phosphoserine is dephosphorylated
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT1]
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT1]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2]
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2]
Oxidative decarboxylation of branched-chain alpha-keto acids
a-ketoisocaproate, a-keto b-methylvalerate, or a-ketoisovalerate + CoA + NAD+ => isovaleryl-CoA, a-methylbutyryl-CoA, or isobuyryl-CoA + CO2 + NADH + H+
isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2
beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA]
beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O <=> beta-methylcrotonyl-CoA + ATP + CO2 [MCCA]
beta-methylglutaconyl-CoA + H2O <=> beta-hydroxy-beta-methylglutaryl-CoA
alpha-methylbutyryl-CoA + FAD => tiglyl-CoA + FADH2
tiglyl-CoA + H2O <=> alpha-methyl-beta-hydroxybutyryl-CoA
alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ <=> alpha-methylacetoacetyl-CoA + NADH + H+
alpha-methylacetoacetyl-CoA + NADH + H+ <=> alpha-methyl-beta-hydroxybutyryl-CoA + NAD+
alpha-methyl-acetoacetyl-CoA + CoA => propionyl-CoA + acetyl-CoA
isobutyryl-CoA + FAD => methacrylyl-CoA + FADH2
methacrylyl-CoA + H2O <=> beta-hydroxyisobutyryl-CoA
beta-hydroxyisobutyryl-CoA + H2O => beta-hydroxyisobutyrate + CoA
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+
methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+
methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+
histidine => urocanate + NH4+
urocanate + H2O => 4-imidazolone-5-propionate
4-imidazolone-5-propionate + H2O => N-formiminoglutamate + 2H+
N-formiminoglutamate + tetrahydrofolate => glutamate + 5-formiminotetrahydrofolate
Histidine is decarboxylated to histamine
lysine + alpha-ketoglutarate +NADPH + H+ => saccharopine + NADP+ + H2O
saccharopine + NAD+ + H2O => alpha-aminoadipic semialdehyde + glutamate + NADH + H+
alpha-aminoadipic semialdehyde + NAD+ => alpha-aminoadipate + NADH + H+
alpha-aminoadipate + alpha-ketoglutarate <=> alpha-ketoadipate + glutamate
alpha-ketoadipate + glutamate <=> alpha-aminoadipate + alpha-ketoglutarate
2-oxoglutarate [mitochondrial matrix] + 2-oxoadipate [cytosol] <=> 2-oxoglutarate [cytosol] + 2-oxoadipate [mitochondrial matrix]
Exchange of cytosolic 2-oxoadipate for mitochondrial 2-oxoglutarate
Oxidative decarboxylation of alpha-ketoadipate to glutaryl CoA by alpha-ketoglutarate dehydrogenase
alpha-ketoadipate + CoASH + NAD+ => glutaryl-CoA + CO2 + NADH + H+
glutaryl-CoA + FAD => crotonyl-CoA + FADH2 + CO2
phenylalanine + pyruvate => 3-(indol-3-yl)pyruvate + alanine
4a-hydroxytetrahydrobiopterin => q-dihydrobiopterin + H2O
q-dihydrobiopterin + NADH + H+ => tetrahydrobiopterin + NAD+
tyrosine + alpha-ketoglutarate <=> p-hydroxyphenylpyruvate + glutamate
p-hydroxyphenylpyruvate + glutamate <=> tyrosine + alpha-ketoglutarate
p-hydroxyphenylpyruvate + O2 => homogentisate + CO2
homogentisate + O2 => maleylacetoacetate
maleylacetoacetate => fumarylacetoacetate
fumarylacetoacetate => fumarate + acetoacetate
proline => L-1-pyrroline-5-carboxylate
L-1-pyrroline-5-carboxylate <=> L-glutamate gamma-semialdehyde
L-glutamate gamma-semialdehyde + NAD+ => glutamate + NADH + H+
tryptophan + O2 => N-formylkynurenine [TDO]
tryptophan + O2 => N-formylkynurenine [IDO]
tryptophan + O2 => N-formylkynurenine [IDO2]
N-formylkynurenine + H2O => kynurenine + formate
kynurenine + pyruvate => 4-(2-aminophenyl)-2,4-dioxobutanoic acid + alanine [CCBL2]
4-(2-aminophenyl)-2,4-dioxobutanoate => kynurenate + H2O [cytosolic]
4-(2-aminophenyl)-2,4-dioxobutanoate => kynurenic acid + H2O [cytosolic]
kynurenine + O2 + NADPH + H+ => 3-hydroxykynurenine + NADP+ + H2O
3-hydroxykynurenine + H2O => 3-hydroxyanthranilate + alanine
3-hydroxyanthranilate + O2 => 2-amino-3-carboxymuconate semialdehyde
2-amino-3-carboxymuconate semialdehyde => 2-aminomuconate semialdehyde + CO2
2-aminomuconate semialdehyde + NAD+ + H2O => aminomuconate + NADH + H+
kynurenine + 2-oxoglutarate => 4-(2-aminophenyl)-2,4-dioxobutanoic acid + glutamate
4-(2-aminophenyl)-2,4-dioxobutanoate => kynurenate + H2O [mitochondrial]
4-(2-aminophenyl)-2,4-dioxobutanoate => kynurenic acid + H2O [mitochondrial]
glutamate + acetyl CoA => N-acetyl glutamate + CoA
2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate
2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial]
carbamoyl phosphate + ornithine => citrulline + orthophosphate
ATP + aspartate + citrulline <=> argininosuccinate + AMP + pyrophosphate
argininosuccinate <=> fumarate + arginine
arginine + H2O => ornithine + urea [ARG1]
arginine + H2O => ornithine + urea [ARG2]
ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic)
trimethyllysine + alpha-ketoglutarate + O2 => beta-hydroxy-trimethyllysine + succinate + CO2
beta-hydroxy-trimethyllysine => gamma-butyrobetaine aldehyde + glycine
gamma-butyrobetaine semialdehyde + NAD+ => gamma-butyrobetaine + NADH + H+
gamma-butyrobetaine + alpha-ketoglutarate + O2 => carnitine + succinate + CO2
arginine + glycine => ornithine + guanidoacetate
guanidinoacetate + S-adenosylmethionine => creatine + S-adenosylhomocysteine
creatine + ATP => phosphocreatine + ADP [CKB,CKM]
creatine + ATP => phosphocreatine + ADP [CK octamer]
phosphocreatine + H2O => creatinine + orthophosphate
Creatine transport across the plasma membrane
Arginine<=>Agmatine+CO2
Agmatine + H2O <=> putrescine + urea
ornithine => putrescine + CO2
S-Adenosyl methionine <=> Decarboxylated-Adenosyl methionine + CO2
Putrescine + dc-Adenosyl methionine => Spermidine + 5'-methylthioadenosine
dc-Adenosyl methionine + Spermidine => Spermine + 5'-methylthioadenosine
Spermidine => N-acetylated spermidine
N-acetylated spermidine (cytosol)=>N-acetylated spermidine (peroxisomal)
N-acetylspermidine is oxidized to putrescine
Spermine => N-acetylated spermine
N-acetylated spermine (cytosol)=>N-acetylated spermine (peroxisomal)
N-acetylspermine is oxidised to spermidine
Spermine is oxidized to spermidine
MTA is cleaved and phosphorylated
Methylthio-ribose-P = Methylthio-ribulose-P
Methylthio-ribulose-P = Methylthio-ribose-P
Dehydration of methylthio-ribulose-P
Acireductone is created
Acireductone is oxidized to MOB
Transamination of MOB to methionine
Antizyme OAZ binds to Ornithine decarboxylase
26S proteosome degrades ODC holoenzyme complex
Antizyme inhibitor binds to OAZ and stablizes ODC complex
NQO1 interaction with ODC
Tyrosine is hydroxylated to dopa
Dopa is decarboxylated to dopamine
Dopamine translocates to a secretory vesicle
Dopamine is oxidised to noradrenaline
Noradrenaline translocates to the cytosol
Noradrenaline is converted to adrenaline
Iodide is taken up by thyroid epithelial cells
Iodide is organified
Tyrosine is monoiodinated
Monoiodinated tyrosine can be deiodinated
Tyrosine is diiodinated
Diiodinated tyrosine can be deiodinated
DIT and MIT combine to form triiodothyronine
Two DITs combine to form thyroxine
Thyroxine is deiodinated to triiodothyronine
Thyroxine is deiodinated to reverse triiodothyronine (RT3)
Tryptophan is hydroxylated
Decarboxylation of 5-hydroxytryptophan forms serotonin
N-acetylation of serotonin
Methylation of N-acetyl-5-HT to form melatonin
Synthesis of glyoxylate from hydroxyproline
glycine + O2 => glyoxylate + H2O2 + NH4+
glyoxylate + alanine => glycine + pyruvate [peroxisome]
glyoxylate + alanine => glycine + pyruvate [mitochondrial matrix]
glyoxylate + NADPH + H+ => glycolate + NADP+
glycolate + O2 => glyoxylate + H2O2
Conversion of glyoxylate to oxalate
SAM is sythesized from methionine's reaction with ATP
S-adenoylhomocysteine is hydrolyzed
Remethylation of homocysteine via betaine degradation
Cystathionine is formed from homocysteine and serine
Cleavage of cystathionine into cysteine
Glutamate and cysteine combine
A dipeptidase cleaves cysteinylglycine
Homocysteine is degraded to oxobutanoate and H2S
Cysteine is degraded to serine and H2S
Cysteine is oxidized
CSAD decarboxylates 3-sulfinoalanine to hypotaurine
Oxidation of hypotaurine to taurine
Excess homocysteine yields homolanthionine and H2S
Excess cysteine yields lanthionine and H2S
SQR oxidizes sulfide to bound persulfide
Persulfide sulfur is dioxygenated
Persulfide sulfur is transferred onto sulfite
Thiosulfate can transfer its sulfate to glutathione
Sulfite is oxidized to sulfate
Amino acid and derivative metabolism
Metabolism of amino acids and derivatives
Amino acid synthesis and interconversion (transamination)
Serine biosynthesis
Branched-chain amino acid catabolism
Histidine catabolism
Lysine catabolism
Phenylalanine and tyrosine catabolism
Proline catabolism
Tryptophan catabolism
Urea cycle
Urea synthesis
Carnitine synthesis
Creatine metabolism
Metabolism of polyamines
Agmatine biosynthesis
Interconversion of polyamines
Methionine salvage pathway
Regulation of ornithine decarboxylase (ODC)
Amine-derived hormones
Catecholamine biosynthesis
Thyroxine biosynthesis
Regulation of thyroid hormone activity
Serotonin and melatonin biosynthesis
Glyoxylate metabolism
Sulfur amino acid metabolism
Cysteine formation from homocysteine
Degradation of cysteine and homocysteine
Sulfide oxidation to sulfate