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Information on EC 1.1.99.2 - L-2-hydroxyglutarate dehydrogenase
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1.1.99.2 L-2-hydroxyglutarate dehydrogenaseSpecify your search results
Word Map
- 1.1.99.2
-
aciduria
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neurometabolic
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ataxia
-
l-2-hga
-
subcortical
-
psychomotor
-
oncometabolite
-
neuroradiological
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terrier
-
extrapyramidal
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macrocephaly
- d2hgdh
- leukoencephalopathy
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hyperintensity
- medicine
The enzyme appears in viruses and cellular organisms
Synonyms
l2hgdh, l-2-hydroxyglutarate dehydrogenase, 2-hydroxyglutarate dehydrogenase, l-2-hg dehydrogenase, l-2hg dehydrogenase, l-2hgdh, l-2-hgdh, alpha-ketoglutarate reductase, l-2-hydroxyglutaric acid dehydrogenase, alpha-hydroxyglutarate dehydrogenase, 
more
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
2-hydroxyglutarate dehydrogenase
alpha-hydroxyglutarate dehydrogenase
-
-
-
-
alpha-hydroxyglutarate dehydrogenase (NAD+ specific)
-
-
-
-
alpha-hydroxyglutarate oxidoreductase
-
-
-
-
alpha-ketoglutarate reductase
-
-
-
-
dehydrogenase, 2-hydroxyglutarate
-
-
-
-
hydroxyglutaric dehydrogenase
-
-
-
-
L-2-hydroxyglutarate dehydrogenase
L-2-hydroxyglutaric acid dehydrogenase
L-alpha-hydroxyglutarate dehydrogenase
-
-
-
-
L-alpha-hydroxyglutarate:NAD+ 2-oxidoreductase
-
-
-
-
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
(S)-2-hydroxyglutarate + acceptor = 2-oxoglutarate + reduced acceptor
-
-
-
-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
oxidation
redox reaction
-
-
-
-
reduction
oxidation
-
-
-
-
reduction
-
-
-
-
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SYSTEMATIC NAME
IUBMB Comments
(S)-2-hydroxyglutarate:acceptor 2-oxidoreductase
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CAS REGISTRY NUMBER
COMMENTARY
9028-80-2
-
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
(R)-2-hydroxyglutarate + 2,6-dichlorophenolindophenol
2-oxoglutarate + reduced 2,6-dichlorophenolindophenol
-
3.7% activity compared to (S)-2-hydroxyglutarate
-
-
?
(R)-2-hydroxyglutarate + NAD+
2-oxoglutarate + NADH
-
about 10% of the activity with (S)-2-hydroxyglutarate
-
-
ir
(S)-2-hydroxyglutarate + 2,6-dichlorophenolindophenol
2-oxoglutarate + reduced 2,6-dichlorophenolindophenol
-
-
-
-
?
2-hydroxy-n-hexanoate + NAD+
2-oxo-n-hexanoate + NADH + H+
-
at 12.4% of the activity with 2-oxoglutarate
-
-
?
2-hydroxy-n-pentanoate + NAD+
2-oxo-n-pentanoate + NADH + H+
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at 10.7% of the activity with 2-oxoglutarate
-
-
?
2-hydroxybutanoate + NAD+
2-oxobutanoate + NADH
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at 9.6% of the activity with 2-oxoglutarate
-
-
?
2-hydroxymalonate + NAD+
oxomalonate + NADH + H+
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at 29.3% of the activity with 2-oxoglutarate
-
-
?
L-2-hydroxyglutarate + iodonitrotetrazolium chloride
2-oxoglutarate + reduced iodonitrotetrazolium chloride
activity assay
-
-
?
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
-
-
-
-
?
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
-
-
-
?
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria
-
-
?
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
-
-
-
-
?
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
-
enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria
-
-
?
(S)-2-hydroxyglutarate + FAD
2-oxoglutarate + FADH2
-
enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria
-
-
?
(S)-2-hydroxyglutarate + NAD+
2-oxoglutarate + NADH + H+
-
in the reverse reaction almost 100% production of the S-isomer of 2-hydroxyglutarate
-
-
r
additional information
?
-
-
high levels of the enzyme is produced at the late stage of cultivation in the presence of citrate and with limited aeration
-
-
?
additional information
?
-
-
the enzyme is not active in the presence of NAD+, NADP+, cytochrome c or O2, as well as glycolate, D-lactate and L-lactate
-
-
?
additional information
?
-
-
mutations (K81E, E176D, DELTA-exon9) found in patients with L-2-hydroxyglutaric aciduria supress L-2-hydroxyglutarate dehydrogenase activity. L-2-hydroxyglutartic aciduria is due to a deficiency in L-2-hydroxyglutarate dehydrogenase
-
-
?
additional information
?
-
-
no activity with 3-acetylpyridine adenine dinucleotide or NADP+
-
-
?
additional information
?
-
-
no activity with D-2-hydroxyglutarate, L-malate, and L-lactate
-
-
?
additional information
?
-
-
no activity with 3-acetylpyridine adenine dinucleotide or NADP+
-
-
?
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
(S)-2-hydroxyglutarate + FAD
2-oxoglutarate + FADH2
-
enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria
-
-
?
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria
-
-
?
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
-
enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria
-
-
?
additional information
?
-
-
high levels of the enzyme is produced at the late stage of cultivation in the presence of citrate and with limited aeration
-
-
?
additional information
?
-
-
mutations (K81E, E176D, DELTA-exon9) found in patients with L-2-hydroxyglutaric aciduria supress L-2-hydroxyglutarate dehydrogenase activity. L-2-hydroxyglutartic aciduria is due to a deficiency in L-2-hydroxyglutarate dehydrogenase
-
-
?
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
additional information
-
activity is not affected by 0.01 mM Co2+ or 0.01 mM Zn2+
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Brain Diseases
A novel compound heterozygous mutation in a Chinese boy with L-2-hydroxyglutaric aciduria: a case study.
Brain Neoplasms
L-2-hydroxyglutaric aciduria and brain tumors in children with mutations in the L2HGDH gene: neuroimaging findings.
Brain Neoplasms
Mutational analysis of D2HGDH and L2HGDH in brain tumours without IDH1 or IDH2 mutations.
Carcinoma
Biochemical and Epigenetic Insights into L-2-Hydroxyglutarate, a Potential Therapeutic Target in Renal Cancer.
Carcinoma
Epigenetic dysregulation by aberrant metabolism in renal cell carcinoma can be reversed with Ascorbic acid.
Carcinoma, Renal Cell
Biochemical and Epigenetic Insights into L-2-Hydroxyglutarate, a Potential Therapeutic Target in Renal Cancer.
Carcinoma, Renal Cell
Epigenetic dysregulation by aberrant metabolism in renal cell carcinoma can be reversed with Ascorbic acid.
Drug Resistant Epilepsy
White matter abnormalities in an adult patient with l-2-hydroxyglutaric aciduria.
Intellectual Disability
White matter abnormalities in an adult patient with l-2-hydroxyglutaric aciduria.
l-2-hydroxyglutarate dehydrogenase deficiency
Adult manifestation of L-2-hydroxyglutarate dehydrogenase deficiency by a novel mutation.
l-2-hydroxyglutarate dehydrogenase deficiency
Familial hyperlysinaemia due to L-lysine alpha-ketoglutarate reductase deficiency: results of attempted treatment.
l-2-hydroxyglutarate dehydrogenase deficiency
L2hgdh Deficiency Accumulates l-2-Hydroxyglutarate with Progressive Leukoencephalopathy and Neurodegeneration.
Leukoencephalopathies
L2hgdh Deficiency Accumulates l-2-Hydroxyglutarate with Progressive Leukoencephalopathy and Neurodegeneration.
Leukoencephalopathies
Metabolite proofreading, a neglected aspect of intermediary metabolism.
Neoplasms
Another small molecule in the oncometabolite mix: L-2-Hydroxyglutarate in kidney cancer.
Neoplasms
Biochemical and Epigenetic Insights into L-2-Hydroxyglutarate, a Potential Therapeutic Target in Renal Cancer.
Neoplasms
L-2-Hydroxyglutarate: an epigenetic modifier and putative oncometabolite in renal cancer.
Neuroinflammatory Diseases
L2hgdh Deficiency Accumulates l-2-Hydroxyglutarate with Progressive Leukoencephalopathy and Neurodegeneration.
Starvation
An L-2-hydroxyglutarate biosensor based on specific transcriptional regulator LhgR.
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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TURNOVER NUMBER [1/s]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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Ki VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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pH RANGE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
5 - 8.5
-
pH 5.0: about 45% of maximal activity, pH 8.5: 60% of maximal activity, reaction with alpha-oxoglutarate and NADH
7.3 - 9.3
7.3 - 9.3
-
the enzyme is active over a wide pH range (7.3-9.3) with no clear optimum
7.3 - 9.3
-
the enzyme is active over a wide pH range (7.3-9.3) with no clear optimum
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TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
-
high levels of the enzyme is produced at the late stage of cultivation in the presence of citrate and with limited aeration
brenda
additional information
additional information
-
no activity in heart, brain and skeletal muscle, cultured human skin fibroblasts and transformed lymphocyte
brenda
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LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
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GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
malfunction
-
L-2-hydroxyglutaric aciduria is a rare inborn error of metabolism in the enzyme is defective
metabolism
metabolism
L-2-hydroxyglutarate dehydrogenase might be regarded as an enzyme of metabolic repair
metabolism
-
2-hydroxyglutarate dehydrogenase is involved exclusively in lysine degradation. 2-hydroxyglutarate dehydrogenase act as electron donors to the ubiquinol pool via an electron-transfer flavoprotein/electron-transfer flavoprotein:ubiquinone oxidoreductase-mediated route
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UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable).
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable). L2HDH_ACCSB
Accumulibacter sp. (strain BA-93)
388
0
40540
Swiss-Prot
-
L2HDH_ARATH
483
0
53526
Swiss-Prot
other Location (Reliability: 5)
L2HDH_BOVIN
463
0
51022
Swiss-Prot
Mitochondrion (Reliability: 2)
L2HDH_CAEBR
434
0
48359
Swiss-Prot
other Location (Reliability: 5)
L2HDH_CAEEL
433
0
47901
Swiss-Prot
other Location (Reliability: 5)
L2HDH_DICDI
446
0
49908
Swiss-Prot
other Location (Reliability: 3)
L2HDH_HUMAN
463
0
50316
Swiss-Prot
Mitochondrion (Reliability: 1)
L2HDH_INDAL
Indibacter alkaliphilus (strain CCUG 57479 / KCTC 22604 / LW1)
399
0
44131
Swiss-Prot
-
L2HDH_MOUSE
464
0
50899
Swiss-Prot
Mitochondrion (Reliability: 3)
L2HDH_NEMVE
456
0
50175
Swiss-Prot
Mitochondrion (Reliability: 1)
L2HDH_PONAB
419
0
45538
Swiss-Prot
Mitochondrion (Reliability: 2)
A0A0B2QQV7_GLYSO
447
0
49218
TrEMBL
Mitochondrion (Reliability: 3)
A0A6J4HBK0_9ACTN
464
0
46888
TrEMBL
-
A0A6J8EGT8_MYTCO
466
0
51837
TrEMBL
Mitochondrion (Reliability: 2)
A0A6J4IHY5_9PROT
468
0
49379
TrEMBL
-
A0A212KT15_9CHLA
404
0
44285
TrEMBL
-
A0A0A1U537_ENTIV
1068
0
115383
TrEMBL
other Location (Reliability: 4)
G2KUM4_FRUST
Fructilactobacillus sanfranciscensis (strain TMW 1.1304)
339
0
37228
TrEMBL
-
A0A6J4J7V5_9ACTN
457
0
46556
TrEMBL
-
A0A2I0B5I7_9ASPA
423
0
45678
TrEMBL
Mitochondrion (Reliability: 4)
A0A812BVC7_SEPPH
161
1
18480
TrEMBL
Mitochondrion (Reliability: 3)
A0A6J4S268_9SPHN
184
0
18582
TrEMBL
-
A0A081S3K5_9ARCH
437
1
49257
TrEMBL
-
A0A3B0R963_9ZZZZ
180
0
19091
TrEMBL
other Location (Reliability: 2)
A0A6J4JM71_9ACTN
360
0
36025
TrEMBL
-
Q1LPA0_CUPMC
Cupriavidus metallidurans (strain ATCC 43123 / DSM 2839 / NBRC 102507 / CH34)
368
0
38866
TrEMBL
-
A0A6J4H073_9PROT
477
0
50216
TrEMBL
-
A0A7R8H004_LEPSM
680
3
78394
TrEMBL
Secretory Pathway (Reliability: 1)
B0E8T6_ENTDS
Entamoeba dispar (strain ATCC PRA-260 / SAW760)
244
0
27236
TrEMBL
other Location (Reliability: 4)
K4R8U7_STRDJ
Streptomyces davaonensis (strain DSM 101723 / JCM 4913 / KCC S-0913 / 768)
402
0
43106
TrEMBL
-
A0A3B0VY29_9ZZZZ
459
0
50877
TrEMBL
other Location (Reliability: 2)
B3QTE3_CHLT3
Chloroherpeton thalassium (strain ATCC 35110 / GB-78)
399
1
43544
TrEMBL
-
B7P885_IXOSC
153
0
17038
TrEMBL
other Location (Reliability: 3)
A0A087S084_9ARCH
437
1
49257
TrEMBL
-
A0A3B0SGG9_9ZZZZ
472
0
50357
TrEMBL
other Location (Reliability: 2)
G7KP59_MEDTR
458
0
50204
TrEMBL
Chloroplast (Reliability: 3)
A0A3G7N3I4_9PSED
468
0
49865
TrEMBL
-
A0A3B0TXK8_9ZZZZ
478
0
49134
TrEMBL
other Location (Reliability: 2)
B3E6A9_TRIL1
Trichlorobacter lovleyi (strain ATCC BAA-1151 / DSM 17278 / SZ)
403
0
44528
TrEMBL
-
A0A8B6GWL8_MYTGA
462
0
51440
TrEMBL
Mitochondrion (Reliability: 3)
A0A6J4P1X7_9RHOB
uncultured Rubellimicrobium sp
470
0
49518
TrEMBL
-
A0A6J4PUR6_9RHOB
uncultured Rubellimicrobium sp
119
0
12757
TrEMBL
-
E0VP15_PEDHC
461
0
51929
TrEMBL
other Location (Reliability: 3)
A0A2P6RH41_ROSCH
426
0
46795
TrEMBL
Mitochondrion (Reliability: 4)
A0A3G7CBX3_9PSED
472
0
51924
TrEMBL
-
A0A3B0UTX0_9ZZZZ
471
0
49860
TrEMBL
other Location (Reliability: 2)
G8S2J8_ACTS5
Actinoplanes sp. (strain ATCC 31044 / CBS 674.73 / SE50/110)
396
0
42935
TrEMBL
-
A0A812C034_SEPPH
293
0
32570
TrEMBL
other Location (Reliability: 1)
A0A087RU82_9ARCH
437
1
49257
TrEMBL
-
A0A3B0THP8_9ZZZZ
765
0
85045
TrEMBL
other Location (Reliability: 1)