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Metabolism of amino acids and derivatives
Amino acid transport across the plasma membrane
cytosol
mitochondrial matrix
peroxisomal matrix
L-Alanine
Pyruvate
GPT dimer
GPT2 dimer
Oxaloacetate
GOT1 dimer
L-Aspartate
GOT2 dimer
L-Glutamate 5-semialdehyde
OAT hexamer
P5CS dimers
(S)-1-Pyrroline-5-carboxylate
L-Proline
PYCR1 decamer
pyrophosphate
L-Asparagine
AMP
ASNS dimer
NH4+
GLUD1 hexamer
GTP
NAD(P)H
NAD(P)+
ATP
Orthophosphate
GLUL decamer
L-Glutamine
GLS dimers
BCAT1 dimer
2-oxoglutarate
Leu, Ile, Val
a-ketoisocaproate, a-keto b-methylvalerate, a-ketoisovalerate
BCAT2 dimer
isovaleryl-CoA, a-methylbutyryl-CoA, isobutyryl-CoA
branched-chain alpha-ketoacid dehydrogenase complex
isovaleryl-CoA
beta-methylcrotonyl-CoA
IVD tetramer
beta-methylglutaconyl-CoA
ADP
methylcrotonyl-CoA carboxylase complex
beta-hydroxy-beta-methylglutaryl-CoA
AUH hexamer
alpha-methylbutyryl-CoA
tiglyl-CoA
ACADSB tetramer
FAD
alpha-methyl-beta-hydroxybutyryl-CoA
enoyl-CoA hydratase
alpha-methylacetoacetyl-CoA
HSD17B10 tetramer
propionyl-CoA
ACAT1 tetramer
isobutyryl-CoA
methacrylyl-CoA
ACAD8 tetramer
FADH2
beta-hydroxyisobutyryl-CoA
3-hydroxyisobutyryl-coenzyme A hydrolase
2-Methyl-3-oxopropanoate
HIBADH tetramer
ALDH6A1
L-Histidine
Urocanate
Histidine ammonia-lyase
H2O
4-Imidazolone-5-propanoate
urocanate hydratase
N-Formimino-L-glutamate
imidazolonepropionase (probable)
Tetrahydrofolate
5-formiminotetrahydrofolate
FTCD octamer
L-Lysine
saccharopine
AASS tetramer
H+
NADP+
L-2-Aminoadipate 6-semialdehyde
L-2-Aminoadipate
ALDH7A1 tetramer
AADAT dimer
alpha-ketoadipate
SLC25A21
glutaryl-CoA
alpha-ketoglutarate dehydrogenase complex
CoA-SH
crotonyl-CoA
GCDH tetramer
L-Phenylalanine
Tetrahydrobiopterin
4a-hydroxytetrahydrobiopterin
L-Tyrosine
PAH tetramer
q-dihydrobiopterin
PCBD1 tetramer
QDPR dimer
3-(4-Hydroxyphenyl)pyruvate
TAT dimer
L-Glutamate
Homogentisate
HPD dimer
4-Maleylacetoacetate
HGD hexamer
4-Fumarylacetoacetate
GSTZ1 dimer
Acetoacetate
FAH dimer
proline dehydrogenase
ALDH4A1 dimer
L-Tryptophan
Formylkynurenine
TDO2 tetramer
Formate
L-Kynurenine
AFMID
3-Hydroxy-L-kynurenine
KMO
3-Hydroxyanthranilate
KYNU dimer
2-Amino-3-carboxymuconate semialdehyde
HAAO
2-Aminomuconate semialdehyde
ACMSD dimer
2-Aminomuconate
aminomuconate-semialdehyde dehydrogenase
NADH
N-Acetyl-L-glutamate
NAGS
Acetyl-CoA
HCO3-
Carbamoyl phosphate
CPS1
L-Citrulline
OTC trimer
Mitochondrial ornithine transporters
N-(L-Arginino)succinate
ASS1 tetramer
L-Arginine
ASL tetramer
Fumarate
Urea
ARG1 trimer
ARG2 trimer
L-Ornithine
N6,N6,N6-Trimethyl-L-lysine
3-Hydroxy-N6,N6,N6-trimethyl-L-lysine
TMLHE dimer
CO2
2-Oxoglutarate
4-Trimethylammoniobutanal
SHMT1 tetramer
4-Trimethylammoniobutanoate
ALDH9A1 tetramer
Carnitine
Succinate
BBOX1 dimer
Glycine
GATM dimer
Guanidinoacetate
GAMT
Phosphocreatine
CKB, CKM
CK octamers
Creatinine
Agmatine
ADC
AGMAT
ODC
S-Adenosylmethioninamine
AdoMetDC
Spermidine
S-methylthio adenosine
SRM
Spermine
SMS
N-acetylspermidine
SSAT-1
N-acetylspermine
3-acetamidopropanal
Putrescine
PAO-FAD complex
3-aminopropanal
Spermidine Oxidase (SMOX)
OAZ
ODC:OAZ complex
NQO1
ODC:NQO1 complex
26S proteasome
AZIN1
AZIN1 bound OAZ:ODC complex
IDO1
Dopa
TH:Fe++
Dopamine
DDC dimer
secretory granule
Ascorbate
Dehydroascorbate
Noradrenaline
DBH tetramer
Adrenaline
PNMT
S-adenosylhomocysteine
5-hydroxytryptophan
TPH1
5-Hydroxytryptamine
N-acetyl-5-hydroxytryptamine
AANAT
Melatonin
ASMT
S-adenosylmethionine
Iodide
NIS
NADPH
Iodine
TPO
Monoiodotyrosine
DEHAL1:FMN
Diiodotyrosine
3,5,3'-triiodothyronine
Thyroxine
DIO dimer
DIO3
NAD+
beta-hydroxyisobutyrate
4-hydroxy-L-proline
Glyoxylate
glycine
DAO dimer
AGXT dimer
Glycolate
GRHPR
HAO1 tetramer
H2O2
Oxalate
Oxygen
hydrogen iodide
Corticotropin-lipotropin precursor
Corticotropin
PC1:calcium cofactor
Choriogonadotropin subunit beta
Glycoprotein hormones alpha chain
Gonadotropin
Lutropin subunit beta
Lutropin
Thyrotropin subunit beta
Thyrotropin
Follitropin subunit beta
Follitropin
Creatine
SLC6A8
sodium ion
alanine + alpha-ketoglutarate <=> pyruvate + glutamate [GPT]
pyruvate + glutamate <=> alanine + alpha-ketoglutarate [GPT]
pyruvate + glutamate <=> alanine + alpha-ketoglutarate [GPT2]
alanine + alpha-ketoglutarate <=> pyruvate + glutamate [GPT2]
aspartate + alpha-ketoglutarate <=> oxaloacetate + glutamate [GOT1]
oxaloacetate + glutamate <=> aspartate + alpha-ketoglutarate [GOT1]
aspartate + alpha-ketoglutarate <=> oxaloacetate + glutamate [GOT2]
oxaloacetate + glutamate <=> aspartate + alpha-ketoglutarate [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]
glutamate + NH4+ + ATP => glutamine + ADP + orthophosphate [GLUL]
glutamine + H2O => glutamate + NH4+ [GLS]
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]
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
true
4-imidazolone-5-propionate + H2O => N-formiminoglutamate + 2H+
N-formiminoglutamate + tetrahydrofolate => glutamate + 5-formiminotetrahydrofolate
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]
alpha-ketoadipate + CoASH + NAD+ => glutaryl-CoA + CO2 + NADH + H+
glutaryl-CoA + FAD => crotonyl-CoA + FADH2 + CO2
phenylalanine + tetrahydrobiopterin + O2 => tyrosine + 4a-hydroxytetrahydrobiopterin + H2O
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]
N-formylkynurenine + H2O => kynurenine + formate
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+
glutamate + acetyl CoA => N-acetyl glutamate + CoA
2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial]
carbamoyl phosphate + ornithine => citrulline + orthophosphate
ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic)
ATP + aspartate + citrulline <=> argininosuccinate + AMP + pyrophosphate
argininosuccinate <=> fumarate + arginine
arginine + H2O => ornithine + urea [ARG1]
arginine + H2O => ornithine + urea [ARG2]
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
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
Spermine => N-acetylated spermine
N-acetylated spermidine (cytosol)=>N-acetylated spermidine (peroxisomal)
N-acetylated spermine (cytosol)=>N-acetylated spermine (peroxisomal)
N-acetylspermidine is oxidized to putrescine
N-acetylspermine is oxidised to spermidine
Spermine is oxidized to spermidine
Antizyme OAZ binds to Ornithine decarboxylase
NQO1 interaction with ODC
26S proteosome degrades ODC holoenzyme complex
Antizyme inhibitor binds to OAZ and stablizes ODC complex
tryptophan + O2 => N-formylkynurenine [IDO]
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
Tryptophan is hydroxylated
Decarboxylation of 5-hydroxytryptophan forms serotonin
N-acetylation of serotonin
Methylation of N-acetyl-5-HT to form melatonin
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)
Synthesis of glyoxylate from hydroxyproline
glycine + O2 => glyoxylate + H2O2 + NH4+
glyoxylate + alanine => glycine + pyruvate
glyoxylate + NADPH + H+ => glycolate + NADP+
glycolate + O2 => glyoxylate + H2O2
Conversion of glyoxylate to oxalate
Corticotropin cleavage from POMC
Human chorionic gonadotropin (hCG) is a heterodimer
Luteinizing hormone is a heterodimer
Thyrotropin is a heterodimer
Follitropin is a heterodimer
Creatine transport across the plasma membrane
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