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Information on EC 1.1.3.15 - (S)-2-hydroxy-acid oxidase and Organism(s) Homo sapiens and UniProt Accession Q9UJM8

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EC Tree
     1 Oxidoreductases
         1.1 Acting on the CH-OH group of donors
             1.1.3 With oxygen as acceptor
                1.1.3.15 (S)-2-hydroxy-acid oxidase
IUBMB Comments
A flavoprotein (FMN). Exists as two major isoenzymes; the A form preferentially oxidizes short-chain aliphatic hydroxy acids, and was previously listed as EC 1.1.3.1, glycolate oxidase; the B form preferentially oxidizes long-chain and aromatic hydroxy acids. The rat isoenzyme B also acts as EC 1.4.3.2, L-amino-acid oxidase.
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This record set is specific for:
Homo sapiens
UNIPROT: Q9UJM8
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The taxonomic range for the selected organisms is: Homo sapiens
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria, Archaea
Synonyms
l-amino acid oxidase, glycolate oxidase, lactate oxidase, haox1, l-alpha-hydroxy acid oxidase, l-2-hydroxy acid oxidase, lchao, l-lactate monooxygenase, hydroxyacid oxidase 1, (l)-2-haox, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
glycolate oxidase
-
(S)-2-hydroxy-acid oxidase, peroxisomal
-
-
-
-
glycolate oxidase
GOX
-
-
-
-
HAOX1
-
-
-
-
HAOX2
-
-
-
-
HAOX3
-
-
-
-
hydroxy-acid oxidase A
-
-
-
-
hydroxy-acid oxidase B
-
-
-
-
hydroxyacid oxidase A
-
-
-
-
L-2-hydroxy acid oxidase
-
-
-
-
L-alpha-hydroxy acid oxidase
-
-
-
-
long chain L-2-hydroxy acid oxidase 2
-
-
oxidase, L-2-hydroxy acid
-
-
-
-
REACTION
REACTION DIAGRAM
COMMENTARY hide
ORGANISM
UNIPROT
LITERATURE
an (S)-2-hydroxy carboxylate + O2 = a 2-oxo carboxylate + H2O2
show the reaction diagram
the pH affects the kinetic steps of the catalytic mechanism of human glycolate oxidase, the enzyme shows a ping-pong bi-bi kinetic mechanism between pH 6.0 and 10.0, overview. Formation of the enzyme-substrate complex suggests the presence of a protonated group participating in substrate binding
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
redox reaction
-
-
-
-
oxidation
-
-
-
-
reduction
-
-
-
-
oxidative decarboxylation
-
-
-
-
SYSTEMATIC NAME
IUBMB Comments
(S)-2-hydroxy-acid:oxygen 2-oxidoreductase
A flavoprotein (FMN). Exists as two major isoenzymes; the A form preferentially oxidizes short-chain aliphatic hydroxy acids, and was previously listed as EC 1.1.3.1, glycolate oxidase; the B form preferentially oxidizes long-chain and aromatic hydroxy acids. The rat isoenzyme B also acts as EC 1.4.3.2, L-amino-acid oxidase.
CAS REGISTRY NUMBER
COMMENTARY hide
9037-63-2
-
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
2-hydroxyoctanoate + 2,6-dichlorophenolindophenol
2-oxo-octanoate + reduced 2,6-dichlorophenolindophenol
show the reaction diagram
-
-
-
r
glycolate + 2,6-dichlorophenolindophenol
glyoxylate + reduced 2,6-dichlorophenolindophenol
show the reaction diagram
-
-
-
?
glycolate + O2
glyoxylate + H2O2
show the reaction diagram
-
-
-
?
glyoxylate + 2,6-dichlorophenolindophenol
? + reduced 2,6-dichlorophenolindophenol
show the reaction diagram
-
-
-
?
2-hydroxyoctanoate + O2
2-oxooctanoate + H2O2
show the reaction diagram
-
substrates for isoenzymes HAOX1, HAOX2, preferred substrate for isoenzyme HAOX3
-
?
2-hydroxypalmitate + O2
2-oxopalmitate + H2O2
show the reaction diagram
-
substrates for isoenzymes HAOX1, HAOX2
-
?
an (S)-2-hydroxy carboxylate + O2
a 2-oxo carboxylate + H2O2
show the reaction diagram
-
-
-
?
glycolate + 2,6-dichlorophenolindophenol
glyoxylate + reduced 2,6-dichlorophenolindophenol
show the reaction diagram
-
-
-
-
?
glycolate + O2
glyoxylate + H2O2
show the reaction diagram
-
highest activity for isoenzyme HAOX1, no activity for isoenzymes HAOX2, HAOX3
-
?
glyoxylate + 2,6-dichlorophenolindophenol
?
show the reaction diagram
-
-
-
-
?
glyoxylate + O2
? + H2O2
show the reaction diagram
-
substrate for isoenzyme HAOX1
-
?
L-lactate + 2,6-dichlorophenolindophenol
pyruvate + reduced 2,6-dichlorophenolindophenol
show the reaction diagram
-
-
-
-
?
L-mandelate + 2,6-dichlorophenolindophenol
?
show the reaction diagram
-
-
-
-
?
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
glycolate + O2
glyoxylate + H2O2
show the reaction diagram
-
-
-
?
2-hydroxyoctanoate + O2
2-oxooctanoate + H2O2
show the reaction diagram
-
substrates for isoenzymes HAOX1, HAOX2, preferred substrate for isoenzyme HAOX3
-
?
2-hydroxypalmitate + O2
2-oxopalmitate + H2O2
show the reaction diagram
-
substrates for isoenzymes HAOX1, HAOX2
-
?
an (S)-2-hydroxy carboxylate + O2
a 2-oxo carboxylate + H2O2
show the reaction diagram
-
-
-
?
glycolate + O2
glyoxylate + H2O2
show the reaction diagram
-
highest activity for isoenzyme HAOX1, no activity for isoenzymes HAOX2, HAOX3
-
?
glyoxylate + O2
? + H2O2
show the reaction diagram
-
substrate for isoenzyme HAOX1
-
?
COFACTOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
flavin
dependent on
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
4-carboxy-5-dodecylsulfanyl-1,2,3-triazole
-
chloride
inhibits the enzyme at high concentrations
glyoxylate
substrate inhibition at concentrations above 4 mM
2,6-dichlorophenolindophenol
-
inhibition by excess substrate
3-benzyl-4-[[4-(4-fluorophenyl)pyridin-2-yl]methyl]-1H-pyrazole-5-carboxylic acid
-
-
3-ethoxy-4-[[4-(4-fluorophenyl)pyridin-2-yl]methyl]-1H-pyrazole-5-carboxylic acid
-
-
3-ethyl-4-[[4-(4-fluorophenyl)pyridin-2-yl]methyl]-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-(2-phenoxybenzyl)-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-(3-phenoxybenzyl)-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-(4-phenoxybenzyl)-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-(naphthalen-1-ylmethyl)-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-(naphthalen-2-ylmethyl)-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-(quinolin-2-ylmethyl)-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-[2-(trifluoromethoxy)benzyl]-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-[2-(trifluoromethyl)benzyl]-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-[3-(pyridin-3-yl)benzyl]-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-[3-(pyridin-4-yl)benzyl]-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-[3-(trifluoromethyl)benzyl]-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-[4-(trifluoromethoxy)benzyl]-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-[4-(trifluoromethyl)benzyl]-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-[[4'-(trifluoromethoxy)biphenyl-3-yl]methyl]-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-[[4'-(trifluoromethoxy)biphenyl-4-yl]methyl]-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-[[4'-(trifluoromethyl)biphenyl-3-yl]methyl]-1H-pyrazole-5-carboxylic acid
-
-
3-methyl-4-[[4'-(trifluoromethyl)biphenyl-4-yl]methyl]-1H-pyrazole-5-carboxylic acid
-
-
4-(1-benzofuran-2-ylmethyl)-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-(1-benzothiophen-2-ylmethyl)-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-(3-carbamoylbenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-(3-carboxybenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-(3-cyanobenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-(3-fluorobenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-(4'-fluorobiphenyl-3-yl)-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-(4'-fluorobiphenyl-4-yl)-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-(4-carbamoylbenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-(4-carboxybenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-(4-cyanobenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-(4-fluorobenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-(4-fluorophenyl)-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-[(2'-fluorobiphenyl-3-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-[(3'-fluorobiphenyl-3-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-[(4'-carbamoylbiphenyl-3-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-[(4'-carboxybiphenyl-3-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-[(4'-cyanobiphenyl-3-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-[(4'-fluorobiphenyl-2-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-[(4'-fluorobiphenyl-3-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-[(4'-fluorobiphenyl-4-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-[2-(4'-fluorobiphenyl-3-yl)ethyl]-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-[2-(4'-fluorobiphenyl-4-yl)ethyl]-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-[2-(4-fluorophenyl)ethyl]-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-[[4'-(2-amino-2-oxoethyl)biphenyl-3-yl]methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
-
-
4-[[4-(4-fluorophenyl)pyridin-2-yl]methyl]-3-(2-phenylethyl)-1H-pyrazole-5-carboxylic acid
-
-
4-[[4-(4-fluorophenyl)pyridin-2-yl]methyl]-3-(propan-2-yl)-1H-pyrazole-5-carboxylic acid
-
-
4-[[4-(4-fluorophenyl)pyridin-2-yl]methyl]-3-phenyl-1H-pyrazole-5-carboxylic acid
-
-
glycolate
-
inhibition by excess substrate
glyoxylate
-
inhibition by excess substrate
L-Mandelate
-
inhibition by excess substrate
Mandelate
-
-
oxalate
-
-
additional information
-
development of selective inhibitors of Hao2 from screening of a compound library, overview
-
KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.04
2-oxo-octanoate
-
0.141 - 2
glycolate
2.2
glyoxylate
-
0.44 - 0.64
O2
0.033
2,6-dichlorophenolindophenol
-
-
0.045
2-Hydroxyoctanoate
-
isoenzyme HAOX3
0.0056 - 0.32
glycolate
3.4
glyoxylate
-
-
16.5
L-lactate
-
-
1.5
L-Mandelate
-
-
additional information
additional information
-
TURNOVER NUMBER [1/s]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.08
2-oxo-octanoate
-
54.1
flavin
pH 7.0, 30°C, flavin reduction
4.1 - 31.2
glycolate
0.7
glyoxylate
-
3.6 - 15.6
glycolate
0.83
glyoxylate
-
-
0.46
L-lactate
-
-
0.11
L-Mandelate
-
-
additional information
additional information
-
Ki VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
280
chloride
pH 7.0, 25°C
0.078
2,6-dichlorophenolindophenol
-
-
36 - 46
glycolate
12
glyoxylate
-
-
92
L-Mandelate
-
-
IC50 VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.0108
3-benzyl-4-[[4-(4-fluorophenyl)pyridin-2-yl]methyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0012
3-ethoxy-4-[[4-(4-fluorophenyl)pyridin-2-yl]methyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0054
3-ethyl-4-[[4-(4-fluorophenyl)pyridin-2-yl]methyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0095
3-methyl-4-(2-phenoxybenzyl)-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0124
3-methyl-4-(3-phenoxybenzyl)-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0104
3-methyl-4-(4-phenoxybenzyl)-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0226
3-methyl-4-(naphthalen-1-ylmethyl)-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.00812
3-methyl-4-(naphthalen-2-ylmethyl)-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0022
3-methyl-4-(quinolin-2-ylmethyl)-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0023
3-methyl-4-[2-(trifluoromethoxy)benzyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0134
3-methyl-4-[2-(trifluoromethyl)benzyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0315
3-methyl-4-[3-(pyridin-3-yl)benzyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0135
3-methyl-4-[3-(pyridin-4-yl)benzyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.00523
3-methyl-4-[3-(trifluoromethyl)benzyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0073
3-methyl-4-[4-(trifluoromethoxy)benzyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.00912
3-methyl-4-[4-(trifluoromethyl)benzyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.00634
3-methyl-4-[[4'-(trifluoromethoxy)biphenyl-3-yl]methyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0113
3-methyl-4-[[4'-(trifluoromethoxy)biphenyl-4-yl]methyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0083
3-methyl-4-[[4'-(trifluoromethyl)biphenyl-3-yl]methyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0051
3-methyl-4-[[4'-(trifluoromethyl)biphenyl-4-yl]methyl]-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0267
4-(1-benzofuran-2-ylmethyl)-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0083
4-(1-benzothiophen-2-ylmethyl)-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0088
4-(3-carbamoylbenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.003
4-(3-carboxybenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0062
4-(3-cyanobenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.00113
4-(3-fluorobenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.00095
4-(4'-fluorobiphenyl-3-yl)-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0196
4-(4'-fluorobiphenyl-4-yl)-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0008
4-(4-carbamoylbenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0032
4-(4-carboxybenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0006
4-(4-cyanobenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.00714
4-(4-fluorobenzyl)-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0031
4-(4-fluorophenyl)-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0052
4-[(2'-fluorobiphenyl-3-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0205
4-[(3'-fluorobiphenyl-3-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0186
4-[(4'-carbamoylbiphenyl-3-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0032
4-[(4'-carboxybiphenyl-3-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0135
4-[(4'-cyanobiphenyl-3-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0116
4-[(4'-fluorobiphenyl-2-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0166
4-[(4'-fluorobiphenyl-3-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.01125
4-[(4'-fluorobiphenyl-4-yl)methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0095
4-[2-(4'-fluorobiphenyl-3-yl)ethyl]-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0054
4-[2-(4'-fluorobiphenyl-4-yl)ethyl]-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0052
4-[2-(4-fluorophenyl)ethyl]-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0022
4-[[4'-(2-amino-2-oxoethyl)biphenyl-3-yl]methyl]-3-methyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0042
4-[[4-(4-fluorophenyl)pyridin-2-yl]methyl]-3-(2-phenylethyl)-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0021
4-[[4-(4-fluorophenyl)pyridin-2-yl]methyl]-3-(propan-2-yl)-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
0.0011
4-[[4-(4-fluorophenyl)pyridin-2-yl]methyl]-3-phenyl-1H-pyrazole-5-carboxylic acid
Homo sapiens
-
pH and temperature not specified in the publication
3.7
Mandelate
Homo sapiens
-
-
1.8
oxalate
Homo sapiens
-
-
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
7
-
assay at
pH RANGE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
5 - 10
pH profiles, the enzyme shows a ping-pong bi-bi kinetic mechanism between pH 6.0 and 10.0, overview
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
30
assay at
37
assay at
30
-
assay at
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
-
SwissProt
Manually annotated by BRENDA team
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
HAO2 mRNA levels are strongly downregulated in two distinct series of human HCCs, when compared to both normal and cirrhotic peritumoral liver, overview
Manually annotated by BRENDA team
-
distribution of isoenzymes HAOX1, HAOX3
Manually annotated by BRENDA team
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
the enzyme is involved in primary hyperoxaluria, a genetic disorder where overproduction of oxalate results in the formation of kidney stones
evolution
physiological function
L-2-hydroxy acid oxidases are flavin mononucleotide-dependent peroxisomal enzymes, responsible for the oxidation of L-2-hydroxy acids to ketoacids, resulting in the formation of hydrogen peroxide. Oncosuppressive role of HAO2 in hepatocarcinogenesis
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
HAOX1_HUMAN
370
0
40924
Swiss-Prot
other Location (Reliability: 1)
PDB
SCOP
CATH
UNIPROT
ORGANISM
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
41290
-
determined by mass spectrometry. After tag-removal two close bands appear on SDS-PAGE one at 45.287 kDa and one at 41.288 kDa
45290
-
determined by mass spectrometry. After tag-removal two close bands appear on SDS-PAGE one at 45.287 kDa and one at 41.288 kDa
SUBUNIT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
tetramer
-
gel filtration
CRYSTALLIZATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
structures of recombinant GO complexed with sulfate, glyoxylate, and 4-carboxy-5-dodecylsulfanyl-1,2,3-triazole (CDST), determined by X-ray crystallography are reported. A loop region (loop 4), is completely visible when the GO active site contains a small ligand. Lack of electron density for this loop in the GOCDST complex, mimicking a large substrate, indicates that a disordered to ordered transition occurs with substrate binding. Conformational flexibility of Trp110 is responsible for enabling GO to react with R-hydroxy acids. Movement of Trp110 disrupts a hydrogen-bonding network between Trp110, Leu191, Tyr134, and Tyr208. This loss indicates that active site movements are directly linked to changes in the conformation of loop 4
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
H260A
site-directed mutagenesis
H260Q
site-directed mutagenesis
STORAGE STABILITY
ORGANISM
UNIPROT
LITERATURE
-70°C, concentrated solution, stable for several months
-
PURIFICATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
recombinant enzyme by nickel affinity chromatography, ammonium sulfate fractionation and dialysis
using a Ni-NTA-column, gel filtration and ion-exchange chromatography
purified in one step on hydroxyapatite column onto which it is strongly adsorbed. For the initial purification steps, FMN is added to the buffers
-
recombinant isoenzymes
-
CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
expressed in Escherichia coli as a His-tagged fusion protein
expressed in Escherichia coli as a His-tagged fusion protein
-
expression in Escherichia coli, expression in human skin fibroblasts
-
gene HAO2, quantitative RT-PCR enzyme expression analysis
EXPRESSION
ORGANISM
UNIPROT
LITERATURE
the metabolic gene HAO2 is downregulated in hepatocellular carcinoma. Hao2 downregulation is species- and etiology-independent in humans and rodents
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
drug development
in humans the enzyme is a potential drug target for treatment of primary hyperoxaluria, a genetic disorder where overproduction of oxalate results in the formation of kidney stones
diagnostics
the metabolic gene HAO2 is downregulated in hepatocellular carcinoma and predicts metastasis and poor survival. Dysregulation of HAO2 is a very early event in the development of hepatocellular carcinoma and it may represent a useful diagnostic and prognostic marker for human hepatocellular carcinoma
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Jones, J.M.; Morrell, J.C.; Gould, S.J.
Identification and characterization of HAOX1, HAOX2, and HAOX3, three human peroxisomal 2-hydroxy acid oxidases
J. Biol. Chem.
275
12590-12597
2000
Homo sapiens, Rattus sp.
Manually annotated by BRENDA team
Vignaud, C.; Pietrancosta, N.; Williams, E.L.; Rumsby, G.; Lederer, F.
Purification and characterization of recombinant human liver glycolate oxidase
Arch. Biochem. Biophys.
465
410-416
2007
Homo sapiens
Manually annotated by BRENDA team
Murray, M.S.; Holmes, R.P.; Lowther, W.T.
Active site and loop 4 movements within human glycolate oxidase implications for substrate specificity and drug design
Biochemistry
47
2439-2449
2008
Homo sapiens (Q9UJM8), Homo sapiens
Manually annotated by BRENDA team
Pennati, A.; Gadda, G.
Involvement of ionizable groups in catalysis of human liver glycolate oxidase
J. Biol. Chem.
284
31214-31222
2009
Homo sapiens (Q9UJM8), Homo sapiens
Manually annotated by BRENDA team
Barawkar, D.A.; Bandyopadhyay, A.; Deshpande, A.; Koul, S.; Kandalkar, S.; Patil, P.; Khose, G.; Vyas, S.; Mone, M.; Bhosale, S.; Singh, U.; De, S.; Meru, A.; Gundu, J.; Chugh, A.; Palle, V.P.; Mookhtiar, K.A.; Vacca, J.P.; Chakravarty, P.K.; Nargund, R.P.; Wright, S.D.; Roy, S.; Graziano, M.P.; Cully, D.; Ca, C.a.i.
Discovery of pyrazole carboxylic acids as potent inhibitors of rat long chain L-2-hydroxy acid oxidase
Bioorg. Med. Chem. Lett.
22
4341-4347
2012
Homo sapiens, Rattus norvegicus
Manually annotated by BRENDA team
Mattu, S.; Fornari, F.; Quagliata, L.; Perra, A.; Angioni, M.M.; Petrelli, A.; Menegon, S.; Morandi, A.; Chiarugi, P.; Ledda-Columbano, G.M.; Gramantieri, L.; Terracciano, L.; Giordano, S.; Columbano, A.
The metabolic gene HAO2 is downregulated in hepatocellular carcinoma and predicts metastasis and poor survival
J. Hepatol.
64
891-898
2016
Rattus norvegicus (Q07523), Mus musculus (Q9NYQ2), Homo sapiens (Q9NYQ3), Mus musculus C3H (Q9NYQ2), Rattus norvegicus Fischer 344 (Q07523)
Manually annotated by BRENDA team