1.1.99.2: L-2-hydroxyglutarate dehydrogenase
This is an abbreviated version!
For detailed information about L-2-hydroxyglutarate dehydrogenase, go to the full flat file.
Word Map on EC 1.1.99.2
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1.1.99.2
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aciduria
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neurometabolic
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ataxia
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l-2-hga
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subcortical
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psychomotor
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oncometabolite
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neuroradiological
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terrier
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extrapyramidal
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macrocephaly
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d2hgdh
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leukoencephalopathy
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hyperintensity
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medicine
- 1.1.99.2
-
aciduria
-
neurometabolic
-
ataxia
-
l-2-hga
-
subcortical
-
psychomotor
-
oncometabolite
-
neuroradiological
-
terrier
-
extrapyramidal
-
macrocephaly
- d2hgdh
- leukoencephalopathy
-
hyperintensity
- medicine
Reaction
Synonyms
2-hydroxyglutarate dehydrogenase, alpha-hydroxyglutarate dehydrogenase, alpha-hydroxyglutarate dehydrogenase (NAD+ specific), alpha-hydroxyglutarate oxidoreductase, alpha-ketoglutarate reductase, dehydrogenase, 2-hydroxyglutarate, hydroxyglutaric dehydrogenase, L-2-HG dehydrogenase, L-2-HGDH, L-2-hydroxyglutarate dehydrogenase, L-2-hydroxyglutaric acid dehydrogenase, L-2HG dehydrogenase, L-2HGDH, L-alpha-hydroxyglutarate dehydrogenase, L-alpha-hydroxyglutarate:NAD+ 2-oxidoreductase, L2HGDH
ECTree
1.1.99.2 L-2-hydroxyglutarate dehydrogenaseAdvanced search results
results (
results (Substrates Products
Substrates Products on EC 1.1.99.2 - L-2-hydroxyglutarate dehydrogenase
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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
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3.7% activity compared to (S)-2-hydroxyglutarate
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?
(R)-2-hydroxyglutarate + NAD+
2-oxoglutarate + NADH
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about 10% of the activity with (S)-2-hydroxyglutarate
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ir
(S)-2-hydroxyglutarate + 2,6-dichlorophenolindophenol
2-oxoglutarate + reduced 2,6-dichlorophenolindophenol
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-
-
-
?
2-hydroxy-n-hexanoate + NAD+
2-oxo-n-hexanoate + NADH + H+
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at 12.4% of the activity with 2-oxoglutarate
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-
?
2-hydroxy-n-pentanoate + NAD+
2-oxo-n-pentanoate + NADH + H+
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at 10.7% of the activity with 2-oxoglutarate
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-
?
2-hydroxybutanoate + NAD+
2-oxobutanoate + NADH
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at 9.6% of the activity with 2-oxoglutarate
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-
?
2-hydroxymalonate + NAD+
oxomalonate + NADH + H+
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at 29.3% of the activity with 2-oxoglutarate
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-
?
L-2-hydroxyglutarate + iodonitrotetrazolium chloride
2-oxoglutarate + reduced iodonitrotetrazolium chloride
activity assay
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-
?
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
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-
-
-
?
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
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-
-
?
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria
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-
?
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
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-
-
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?
(S)-2-hydroxyglutarate + acceptor
2-oxoglutarate + reduced acceptor
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enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria
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-
?
(S)-2-hydroxyglutarate + FAD
2-oxoglutarate + FADH2
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enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria
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-
?
(S)-2-hydroxyglutarate + NAD+
2-oxoglutarate + NADH + H+
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in the reverse reaction almost 100% production of the S-isomer of 2-hydroxyglutarate
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r
additional information
?
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high levels of the enzyme is produced at the late stage of cultivation in the presence of citrate and with limited aeration
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?
additional information
?
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the enzyme is not active in the presence of NAD+, NADP+, cytochrome c or O2, as well as glycolate, D-lactate and L-lactate
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-
?
additional information
?
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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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?
additional information
?
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no activity with 3-acetylpyridine adenine dinucleotide or NADP+
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?
additional information
?
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no activity with D-2-hydroxyglutarate, L-malate, and L-lactate
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?
additional information
?
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no activity with 3-acetylpyridine adenine dinucleotide or NADP+
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?
