Amino acid and derivative metabolism

Häussinger D. Liver glutamine metabolism. JPEN J Parenter Enteral Nutr. 1990 Jul;14(4 Suppl):56S–62S. doi: 10.1177/014860719001400405. PMID: 2205732.; Owen OE, Reichard GA, Patel MS, Boden G. Energy metabolism in feasting and fasting. Adv Exp Med Biol. 1979;111():169–88. doi: 10.1007/978-1-4757-0734-2_8. PMID: 371355.; Felig P. Amino acid metabolism in man. Annu Rev Biochem. 1975;44():933–55. doi: 10.1146/annurev.bi.44.070175.004441. PMID: 1094924.

Metabolites

4-Trimethylammoniobutanal

Formula: C7H16NO (130.1231826)

CAS ID: 64595-66-0

5'-Methylthioadenosine

Formula: C11H15N5O3S (297.089556)

CAS ID: 2457-80-9

Adenine

Formula: C5H5N5 (135.054493)

CAS ID: 73-24-5

Adenosine

Formula: C10H13N5O4 (267.09674980000005)

CAS ID: 58-61-7

Calcium cation

Formula: Ca (39.962591)

CAS ID: 14127-61-8

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

Fe2+

Formula: Fe (55.934939)

CAS ID: 15438-31-0

Guanidinoacetate

Formula: C3H7N3O2 (117.0538242)

CAS ID: 352-97-6

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

HCO3-

Formula: HCO3 (60.9925696)

CAS ID: 71-52-3

HSO3-

Formula: HSO3 (80.96464160000001)

CAS ID: 15181-46-1

Hydrogen cyanide

Formula: CHN (27.010898599999997)

CAS ID: 74-90-8

L-Carnitine

Formula: C7H15NO3 (161.105188)

CAS ID: 541-15-1

L-Formylkynurenine

Formula: C11H12N2O4 (236.07970319999998)

CAS ID: 1022-31-7

Magnesium cation

Formula: Mg (23.98505)

CAS ID: 22537-22-0

Manganese(2+)

Formula: Mn (54.938046)

CAS ID: 7439-96-5

N-Acetyl-L-glutamate

Formula: C7H11NO5 (189.0637196)

CAS ID: 1188-37-0

N-Acetylserotonin

Formula: C12H14N2O2 (218.1055224)

CAS ID: 1210-83-9

Oxygen

Formula: O2 (31.98983)

CAS ID: 7782-44-7

Sulfate

Formula: H2SO4 (97.9673812)

CAS ID: 7664-93-9

Urea

Formula: CH4N2O (60.0323614)

CAS ID: 57-13-6

Thiocyanate

Formula: CNS (57.975145999999995)

CAS ID: 302-04-5

L-argininium(1+)

Formula: C6H15N4O2 (175.119495)

CAS ID: 17806-42-7

Sulfanegen

Formula: C6H8O6S2 (239.9762308)

CAS ID: 80003-64-1

2-oxo-3-phenylpropanoate

Formula: C9H7O3 (163.0395172)

CAS ID: 620-76-8

formate

Formula: CHO2 (44.997654600000004)

CAS ID: 71-47-6

oxaloacetate(2-)

Formula: C4H2O5 (129.9902242)

CAS ID: 149-63-3

2-oxoglutarate(2-)

Formula: C5H4O5 (144.00587339999998)

CAS ID: 64-15-3

succinate(2-)

Formula: C4H4O4 (116.01095839999999)

CAS ID: 56-14-4

butenedioate

Formula: C4H2O4 (113.99530920000001)

CAS ID: 17013-01-3

pyruvate

Formula: C3H3O3 (87.00821880000001)

CAS ID: 57-60-3

2-oxoglutaramate

Formula: C5H6NO4 (144.0296816)

CAS ID: 18465-19-5

sulfite

Formula: O3S (79.956817)

CAS ID: 14265-45-3

NADPH(4-)

Formula: C21H26N7O17P3 (741.0598016)

CAS ID: 53-57-6

ATP(4-)

Formula: C10H12N5O13P3 (502.9644492)

CAS ID:

NAD(1-)

Formula: C21H26N7O14P2 (662.1012936000001)

CAS ID: 53-84-9

ADP(3-)

Formula: C10H12N5O10P2 (424.0059412)

CAS ID:

NADP(3-)

Formula: C21H25N7O17P3 (740.051977)

CAS ID: 53-59-8

L-lysinium(1+)

Formula: C6H15N2O2 (147.113347)

CAS ID: 56-87-1

hydrosulfide

Formula: HS (32.979896600000004)

CAS ID: 15035-72-0



Enzyme

EC Number name full name note
1.14.11.-
1.13.11.52 indoleamine 2,3-dioxygenase D-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)
1.13.11.53 acireductone dioxygenase (Ni2+-requiring) 1,2-dihydroxy-5-(methylthio)pent-1-en-3-one:oxygen oxidoreductase (formate- and CO-forming)
1.13.11.54 acireductone dioxygenase [iron(II)-requiring] 1,2-dihydroxy-5-(methylthio)pent-1-en-3-one:oxygen oxidoreductase (formate-forming)
1.13.11.6 3-hydroxyanthranilate 3,4-dioxygenase 3-hydroxyanthranilate:oxygen 3,4-oxidoreductase (decyclizing)
1.14.13.9 kynurenine 3-monooxygenase L-kynurenine,NADPH:oxygen oxidoreductase (3-hydroxylating)
1.2.1.38 N-acetyl-gamma-glutamyl-phosphate reductase N-acetyl-L-glutamate-5-semialdehyde:NADP+ 5-oxidoreductase (phosphorylating)
1.2.1.41 glutamate-5-semialdehyde dehydrogenase L-glutamate-5-semialdehyde:NADP+ 5-oxidoreductase (phosphorylating)
1.4.4.2 glycine dehydrogenase (aminomethyl-transferring) glycine:H-protein-lipoyllysine oxidoreductase (decarboxylating, acceptor-amino-methylating)
1.5.1.10 saccharopine dehydrogenase (NADP+, L-glutamate-forming) N6-(L-1,3-dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-glutamate-forming)
1.5.1.2 pyrroline-5-carboxylate reductase L-proline:NAD(P)+ 5-oxidoreductase
1.5.5.2 proline dehydrogenase L-proline:quinone oxidoreductase
1.8.1.4 dihydrolipoyl dehydrogenase protein-N6-(dihydrolipoyl)lysine:NAD+ oxidoreductase
2.1.1.22 carnosine N-methyltransferase S-adenosyl-L-methionine:carnosine N-methyltransferase
2.1.2.10 aminomethyltransferase [protein]-S8-aminomethyldihydrolipoyllysine:tetrahydrofolate aminomethyltransferase (ammonia-forming)
2.1.3.3 ornithine carbamoyltransferase carbamoyl-phosphate:L-ornithine carbamoyltransferase
2.3.1.61 dihydrolipoyllysine-residue succinyltransferase succinyl-CoA:enzyme-N6-(dihydrolipoyl)lysine S-succinyltransferase
2.4.2.28 S-methyl-5'-thioadenosine phosphorylase S-methyl-5'-thioadenosine:phosphate S-methyl-5-thio-alpha-D-ribosyl-transferase
2.5.1.16 spermidine synthase S-adenosyl 3-(methylthio)propylamine:putrescine 3-aminopropyltransferase
2.6.1.1 aspartate transaminase L-aspartate:2-oxoglutarate aminotransferase
2.6.1.13 ornithine aminotransferase L-ornithine:2-oxo-acid aminotransferase
2.6.1.2 alanine transaminase L-alanine:2-oxoglutarate aminotransferase
2.6.1.42 branched-chain-amino-acid transaminase branched-chain-amino-acid:2-oxoglutarate aminotransferase
2.6.1.52 phosphoserine transaminase O-phospho-L-serine:2-oxoglutarate aminotransferase
2.6.1.7 kynurenine-oxoglutarate transaminase L-kynurenine:2-oxoglutarate aminotransferase
2.7.2.8 acetylglutamate kinase ATP:N-acetyl-L-glutamate 5-phosphotransferase
2.8.1.2 3-mercaptopyruvate sulfurtransferase 3-mercaptopyruvate:cyanide sulfurtransferase
3.1.2.4 3-hydroxyisobutyryl-CoA hydrolase 3-hydroxy-2-methylpropanoyl-CoA hydrolase
3.1.3.3 phosphoserine phosphatase O-phosphoserine phosphohydrolase
3.1.3.77 acireductone synthase 5-(methylthio)-2,3-dioxopentyl-phosphate phosphohydrolase (isomerizing)
3.13.2.1 adenosylhomocysteinase S-adenosyl-L-homocysteine hydrolase
3.5.3.1 arginase L-arginine amidinohydrolase
3.7.1.3 kynureninase L-kynurenine hydrolase
4.1.1.17 ornithine decarboxylase L-ornithine carboxy-lyase (putrescine-forming)
4.1.1.50 adenosylmethionine decarboxylase S-adenosyl-L-methionine carboxy-lyase [(5-deoxy-5-adenosyl)(3-aminopropyl)methylsulfonium-salt-forming]
4.2.1.22 cystathionine beta-synthase L-serine hydro-lyase (adding homocysteine; L-cystathionine-forming)
4.2.1.96 4a-hydroxytetrahydrobiopterin dehydratase 4a-hydroxytetrahydrobiopterin hydro-lyase (6,7-dihydrobiopterin-forming)
4.3.1.16 threo-3-hydroxy-L-aspartate ammonia-lyase threo-3-hydroxy-L-aspartate ammonia-lyase (oxaloacetate-forming)
4.3.1.17 L-serine ammonia-lyase L-serine ammonia-lyase (pyruvate-forming)
4.3.1.19 threonine ammonia-lyase L-threonine ammonia-lyase (2-oxobutanoate-forming)
4.3.2.1 argininosuccinate lyase 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
4.4.1.1 cystathionine gamma-lyase L-cystathionine cysteine-lyase (deaminating; 2-oxobutanoate-forming)
6.3.1.2 glutamine synthetase L-glutamate:ammonia ligase (ADP-forming)
6.3.4.16 carbamoyl-phosphate synthase (ammonia) carbon-dioxide:ammonia ligase (ADP-forming, carbamate-phosphorylating)
6.3.5.5 carbamoyl-phosphate synthase (glutamine-hydrolysing) hydrogen-carbonate:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)


Proteins

Protein ID name full name
P00812 CAR1 Arginase
P03965 CPA2 Carbamoyl phosphate synthase arginine-specific large chain
P04076 ARG4 Argininosuccinate lyase
P05150 ARG3 Ornithine carbamoyltransferase
P07991 CAR2 Ornithine aminotransferase
P08432 SPE1 Ornithine decarboxylase
P09368 PUT1 Proline dehydrogenase, mitochondrial
P09624 LPD1 Dihydrolipoyl dehydrogenase, mitochondrial
P19262 KGD2 Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
P21182 SPE2 S-adenosylmethionine decarboxylase proenzyme
P23180 AIM17 Probable oxidoreductase AIM17
P23542 AAT2 Aspartate aminotransferase, cytoplasmic
P25379 CHA1 Catabolic L-serine/threonine dehydratase
P28817 EHD3 Small ribosomal subunit protein mS47
P31373 CYS3 Cystathionine gamma-lyase
P32263 PRO3 Pyrroline-5-carboxylate reductase
P32288 GLN1 Glutamine synthetase
P32582 CYS4 Cystathionine beta-synthase
P32626 UTR4 Enolase-phosphatase E1
P33330 SER1 Phosphoserine aminotransferase
P36007 SRY1 L-threo-3-hydroxyaspartate ammonia-lyase
P38169 BNA4 Kynurenine 3-monooxygenase
P38744 PHS_YEAST Putative pterin-4-alpha-carbinolamine dehydratase
P38891 BAT1 Branched-chain-amino-acid aminotransferase, mitochondrial
P38999 LYS9 Saccharopine dehydrogenase [NADP(+), L-glutamate-forming]
P39954 SAH1 Adenosylhomocysteinase
P42941 SER2 Phosphoserine phosphatase
P47039 BNA3 Probable kynurenine--oxoglutarate transaminase BNA3
P47096 BNA1 3-hydroxyanthranilate 3,4-dioxygenase
P47125 BNA2 Indoleamine 2,3-dioxygenase
P48015 GCV1 Aminomethyltransferase, mitochondrial
P49095 GCV2 Glycine dehydrogenase (decarboxylating), mitochondrial
P52893 ALT1 Alanine aminotransferase, mitochondrial
P53934 CARME_YEAST Carnosine N-methyltransferase
P54885 PRO2 Gamma-glutamyl phosphate reductase
Q01217 ARG5,6 Protein ARG5,6, mitochondrial
Q01802 AAT1 Aspartate aminotransferase, mitochondrial
Q03305 RMT2 Protein arginine N-methyltransferase 2
Q03677 ADI1 Acireductone dioxygenase
Q05979 BNA5 Kynureninase
Q07938 MEU1 S-methyl-5'-thioadenosine phosphorylase
Q08686 TUM1 Thiosulfate sulfurtransferase TUM1
Q12074 SPE3 Spermidine synthase SPE3
Q12447 PAA1 Polyamine N-acetyltransferase 1