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Synonyms
l2hgdh, l-2-hydroxyglutarate dehydrogenase, 2-hydroxyglutarate dehydrogenase, l-2-hg dehydrogenase, l-2hg dehydrogenase, l-2-hgdh, l-2hgdh, l-2-hydroxyglutaric acid dehydrogenase, alpha-ketoglutarate reductase, alpha-hydroxyglutarate dehydrogenase,
more
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(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
-
-
-
-
?
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
(S)-2-hydroxyglutarate + FAD
2-oxoglutarate + FADH2
(S)-2-hydroxyglutarate + iodonitrotetrazolium
2-oxoglutarate + ?
-
-
-
-
?
(S)-2-hydroxyglutarate + NAD+
2-oxoglutarate + NADH + H+
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+
-
at 10.7% of the activity with 2-oxoglutarate
-
-
?
2-hydroxybutanoate + NAD+
2-oxobutanoate + NADH
-
at 9.6% of the activity with 2-oxoglutarate
-
-
?
2-hydroxymalonate + NAD+
oxomalonate + NADH + H+
-
at 29.3% of the activity with 2-oxoglutarate
-
-
?
L-2-hydroxyglutarate + FAD
2-oxoglutarate + FADH2
-
-
-
-
ir
L-2-hydroxyglutarate + iodonitrotetrazolium chloride
2-oxoglutarate + reduced iodonitrotetrazolium chloride
activity assay
-
-
?
L-2-hydroxyglutarate + NAD+
2-oxoglutarate + NADH + H+
additional information
?
-
(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
-
-
-
-
?
(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
(S)-2-hydroxyglutarate + NAD+
2-oxoglutarate + NADH + H+
-
-
-
-
?
(S)-2-hydroxyglutarate + NAD+
2-oxoglutarate + NADH + H+
-
-
-
-
?
L-2-hydroxyglutarate + NAD+
2-oxoglutarate + NADH + H+
-
-
-
-
?
L-2-hydroxyglutarate + NAD+
2-oxoglutarate + NADH + H+
-
-
-
-
r
L-2-hydroxyglutarate + NAD+
2-oxoglutarate + NADH + H+
-
-
-
-
?
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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(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
(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+
-
-
-
-
?
L-2-hydroxyglutarate + FAD
2-oxoglutarate + FADH2
-
-
-
-
ir
L-2-hydroxyglutarate + NAD+
2-oxoglutarate + NADH + H+
-
-
-
-
r
additional information
?
-
(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
-
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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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.
Glioblastoma
LCN2-interacting proteins and their expression patterns in brain tumors.
Glioblastoma
Screen for IDH1, IDH2, IDH3, D2HGDH and L2HGDH mutations in glioblastoma.
Intellectual Disability
White matter abnormalities in an adult patient with l-2-hydroxyglutaric aciduria.
Kidney Neoplasms
Teleological Role of L-2-Hydroxyglutarate Dehydrogenase in the Kidney.
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.
Neoplasms
Screen for IDH1, IDH2, IDH3, D2HGDH and L2HGDH mutations in glioblastoma.
Neoplasms
Teleological Role of L-2-Hydroxyglutarate Dehydrogenase in the Kidney.
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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A140P
-
the mutation is associated with L-2-hydroxyglutaric aciduria
A184V
-
the mutation is associated with L-2-hydroxyglutaric aciduria
A406V
-
the mutation is associated with L-2-hydroxyglutaric aciduria
A62D
-
the mutation is associated with L-2-hydroxyglutaric aciduria
C179R
-
the mutation is associated with L-2-hydroxyglutaric aciduria
DELTA-exon9
-
mutated protein is inactive and abnormally processed
E176G
-
the mutation is associated with L-2-hydroxyglutaric aciduria
E268X
-
the mutation is associated with L-2-hydroxyglutaric aciduria
E336K
-
the mutation is associated with L-2-hydroxyglutaric aciduria
G116D
-
the mutation is associated with L-2-hydroxyglutaric aciduria
G128X
-
the mutation is associated with L-2-hydroxyglutaric aciduria
G156V
-
the mutation is associated with L-2-hydroxyglutaric aciduria
G211D
-
the mutation is associated with L-2-hydroxyglutaric aciduria
G211V
-
the mutation is associated with L-2-hydroxyglutaric aciduria
G260A
-
the mutation is associated with L-2-hydroxyglutaric aciduria
G260V
-
the mutation is associated with L-2-hydroxyglutaric aciduria
G55D
-
the mutation is associated with L-2-hydroxyglutaric aciduria
G57R
-
the mutation is associated with L-2-hydroxyglutaric aciduria
H434P
-
the mutation is associated with L-2-hydroxyglutaric aciduria
H98R
-
the mutation is associated with L-2-hydroxyglutaric aciduria
H98Y
-
the mutation is associated with L-2-hydroxyglutaric aciduria
K246N
-
the mutation is associated with L-2-hydroxyglutaric aciduria
P302L
-
the mutation is associated with L-2-hydroxyglutaric aciduria
P441E
-
the mutation is associated with L-2-hydroxyglutaric aciduria
Q197X
-
the mutation is associated with L-2-hydroxyglutaric aciduria
Q204X
-
the mutation is associated with L-2-hydroxyglutaric aciduria
R251X
-
the mutation is associated with L-2-hydroxyglutaric aciduria
R277X
-
the mutation is associated with L-2-hydroxyglutaric aciduria
R282Q
-
the mutation is associated with L-2-hydroxyglutaric aciduria
R282W
-
the mutation is associated with L-2-hydroxyglutaric aciduria
R335X
-
the mutation is associated with L-2-hydroxyglutaric aciduria
R70X
-
the mutation is associated with L-2-hydroxyglutaric aciduria
S263L
-
the mutation is associated with L-2-hydroxyglutaric aciduria
S263X
-
the mutation is associated with L-2-hydroxyglutaric aciduria
S440Y
-
the mutation is associated with L-2-hydroxyglutaric aciduria
V296E
-
the mutation is associated with L-2-hydroxyglutaric aciduria
Y153X
-
the mutation is associated with L-2-hydroxyglutaric aciduria
Y195C
-
the mutation is associated with L-2-hydroxyglutaric aciduria
Y301X
-
the mutation is associated with L-2-hydroxyglutaric aciduria
Y367C
-
the mutation is associated with L-2-hydroxyglutaric aciduria
E176D
-
mutated protein is inactive and abnormally processed
E176D
-
the mutation is associated with L-2-hydroxyglutaric aciduria
K81E
-
mutated protein is inactive and abnormally processed
K81E
-
the mutation is associated with L-2-hydroxyglutaric aciduria
additional information
A320G, G607T, naturally occurring mutations found in L-2-hydroxyglutaric aciduria affected patients
additional information
-
A320G, G607T, naturally occurring mutations found in L-2-hydroxyglutaric aciduria affected patients
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Suzuki, T.; Uozomi, T.; Beppu, T.
Purification and characterization of a-hydroxyglutarate dehydrogenase from Alcaligenes sp.
Agric. Biol. Chem.
49
2939-2947
1985
Alcaligenes sp.
-
brenda
Jansen, G.A.; Wanders, R.J.A.
L-2-Hydroxyglutarate dehydrogenase: identification of a novel enzyme activity in rat and human liver. Implications for L-2-hydroxyglutaric acidemia
Biochim. Biophys. Acta
1225
53-56
1993
Homo sapiens, Rattus norvegicus
brenda
Achouri, Y.; Noel, G.; Vertommen, D.; Rider, M.H.; Veiga-da-Cunha, M.; van Schaftingen, E.
Identification of a dehydrogenase acting on D-2-hydroxyglutarate
Biochem. J.
381
35-42
2004
Rattus norvegicus
brenda
Rzem, R.; Veiga-da-Cunha, M.; Noel, G.; Goffette, S.; Nassogne, M.C.; Tabarki, B.; Scholler, C.; Marquardt, T.; Vikkula, M.; Van Schaftingen, E.
A gene encoding a putative FAD-dependent L-2-hydroxyglutarate dehydrogenase is mutated in L-2-hydroxyglutaric aciduria
Proc. Natl. Acad. Sci. USA
101
16849-16854
2004
Rattus norvegicus, Homo sapiens (Q9H9P8), Homo sapiens
brenda
Rzem, R.; Van Schaftingen, E.; Veiga-da-Cunha, M.
The gene mutated in L-2-hydroxyglutaric aciduria encodes L-2-hydroxyglutarate dehydrogenase
Biochimie
88
113-116
2006
Homo sapiens
brenda
Penderis, J.; Calvin, J.; Abramson, C.; Jakobs, C.; Pettitt, L.; Binns, M.M.; Verhoeven, N.M.; ODriscoll, E.; Platt, S.R.; Mellersh, C.S.
L-2-hydroxyglutaric aciduria: characterisation of the molecular defect in a spontaneous canine model
J. Med. Genet.
44
334-340
2007
Canis lupus familiaris
brenda
Augoustides-Savvopoulou, P.; Salomons, G.S.; Dotis, J.; Roilides, E.; Leontsini, M.; Jakobs, C.; Panteliadis, C.
Mutation analysis a prerequisite for prenatal diagnosis of L-2-hydroxyglutaric aciduria?
Mol. Genet. Metab.
91
399-401
2007
Homo sapiens
brenda
Kranendijk, M.; Salomons, G.S.; Gibson, K.M.; Aktuglu-Zeybek, C.; Bekri, S.; Christensen, E.; Clarke, J.; Hahn, A.; Korman, S.H.; Mejaski-Bosnjak, V.; Superti-Furga, A.; Vianey-Saban, C.; van der Knaap, M.S.; Jakobs, C.; Struys, E.A.
Development and implementation of a novel assay for L-2-hydroxyglutarate dehydrogenase (L-2-HGDH) in cell lysates: L-2-HGDH deficiency in 15 patients with L-2-hydroxyglutaric aciduria
J. Inherit. Metab. Dis.
32
713-719
2009
Homo sapiens (Q9H9P8)
brenda
Steenweg, M.E.; Jakobs, C.; Errami, A.; van Dooren, S.J.; Adeva Bartolome, M.T.; Aerssens, P.; Augoustides-Savvapoulou, P.; Baric, I.; Baumann, M.; Bonafe, L.; Chabrol, B.; Clarke, J.T.; Clayton, P.; Coker, M.; Cooper, S.; Falik-Zaccai, T.; Gorman, M.; Hahn, A.; Hasanoglu, A.; King, M.D.; de Klerk, H.
An overview of L-2-hydroxyglutarate dehydrogenase gene (L2HGDH) variants: a genotype-phenotype study
Hum. Mutat.
31
380-390
2010
Homo sapiens
brenda
Huedig, M.; Maier, A.; Scherrers, I.; Seidel, L.; Jansen, E.E.; Mettler-Altmann, T.; Engqvist, M.K.; Maurino, V.G.
Plants possess a cyclic mitochondrial metabolic pathway similar to the mammalian metabolic repair mechanism involving malate dehydrogenase and l-2-hydroxyglutarate dehydrogenase
Plant Cell Physiol.
56
1820-1830
2015
Arabidopsis thaliana
brenda
Jansen, G.A.; Wanders, R.J.
L-2-hydroxyglutarate dehydrogenase identification of a novel enzyme activity in rat and human liver. Implications for L-2-hydroxyglutaric acidemia
Biochim. Biophys. Acta
1225
53-56
1993
Homo sapiens, Rattus norvegicus
brenda
Hariharan, V.A.; Denton, T.T.; Paraszcszak, S.; McEvoy, K.; Jeitner, T.M.; Krasnikov, B.F.; Cooper, A.J.
The enzymology of 2-hydroxyglutarate, 2-hydroxyglutaramate and 2-hydroxysuccinamate and their relationship to oncometabolites
Biology
6
E24
2017
Homo sapiens
brenda
Wanders, R.
L-2-hydroxyglutaric aciduria normal L-2-hydroxyglutarate dehydrogenase activity in liver from two new patients
J. Inherit. Metab. Dis.
20
725-726
1997
Homo sapiens
brenda