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(E)-3-methylglutaconyl-1-CoA
?
different co-substrates tested
-
-
?
(R,S)-3-hydroxy-3-methylglutaryl-CoA
?
different co-substrates tested
-
-
?
(S)-3-Hydroxy-3-methylglutaryl-CoA
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
(S)-3-hydroxyglutaryl-CoA
?
3-hydroxybutyryl-CoA
?
different co-substrates tested
-
-
?
3-methylcrotonyl-CoA
?
different co-substrates tested
-
-
?
crotonyl-CoA
?
different co-substrates tested
-
-
?
trans-3-methylglutaconyl-CoA + H2O
(S)-3-hydroxy-3-methylglutaryl-CoA
-
-
-
?
trans-3-methylglutaconyl-CoA + H2O
(S)-3-hydroxymethylglutaryl-CoA
additional information
?
-
(E)-glutaconyl-CoA
?
-
-
-
?
(E)-glutaconyl-CoA
?
-
-
-
?
(E)-glutaconyl-CoA
?
different co-substrates tested
-
-
?
(S)-3-Hydroxy-3-methylglutaryl-CoA
?
-
penultimate step in Leu catabolism
-
-
?
(S)-3-Hydroxy-3-methylglutaryl-CoA
?
-
enzyme is involved in Leu degradation
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
-
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
isovalerate as sole carbon and energy source in Acinetobacter is metabolized via isovaleryl-CoA, oxidative (S)-leucine degradation pathway
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
-
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
isovalerate as sole carbon and energy source in Acinetobacter is metabolized via isovaleryl-CoA, oxidative (S)-leucine degradation pathway
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
-
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
-
-
-
r
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
-
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
-
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
-
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
-
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
-
-
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
-
-
-
r
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
catalyzes fifth step in the leucine degradation pathway
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
fifth step in the leucine degradation pathway
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
four types of the human disease 3-methylglutaconic aciduria distinguished, only form I is due to deficient activity of 3-methylglutaconyl-CoA hydratase leading to accumulation of 3-methylglutaconate
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
leucine degradation pathway, human AUH gene encoding a bifunctional enzyme with RNA-binding activity and enoyl-CoA-hydratase activity
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
-
-
-
?
(S)-3-hydroxyglutaryl-CoA
?
-
-
-
?
(S)-3-hydroxyglutaryl-CoA
?
-
-
-
?
3-methylglutaconyl-CoA
?
-
-
-
?
3-methylglutaconyl-CoA
?
-
-
-
?
trans-3-methylglutaconyl-CoA + H2O
(S)-3-hydroxymethylglutaryl-CoA
-
-
-
?
trans-3-methylglutaconyl-CoA + H2O
(S)-3-hydroxymethylglutaryl-CoA
-
-
-
?
trans-3-methylglutaconyl-CoA + H2O
(S)-3-hydroxymethylglutaryl-CoA
-
-
-
-
?
trans-3-methylglutaconyl-CoA + H2O
(S)-3-hydroxymethylglutaryl-CoA
-
-
-
-
?
additional information
?
-
-
no activity with crotonyl-CoA and methylcrotonyl-CoA
-
-
?
additional information
?
-
-
no activity with crotonyl-CoA and methylcrotonyl-CoA
-
-
?
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(S)-3-Hydroxy-3-methylglutaryl-CoA
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
trans-3-methylglutaconyl-CoA + H2O
(S)-3-hydroxy-3-methylglutaryl-CoA
-
-
-
?
(S)-3-Hydroxy-3-methylglutaryl-CoA
?
-
penultimate step in Leu catabolism
-
-
?
(S)-3-Hydroxy-3-methylglutaryl-CoA
?
-
enzyme is involved in Leu degradation
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
isovalerate as sole carbon and energy source in Acinetobacter is metabolized via isovaleryl-CoA, oxidative (S)-leucine degradation pathway
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
isovalerate as sole carbon and energy source in Acinetobacter is metabolized via isovaleryl-CoA, oxidative (S)-leucine degradation pathway
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
catalyzes fifth step in the leucine degradation pathway
-
-
?
(S)-3-hydroxy-3-methylglutaryl-CoA
trans-3-methylglutaconyl-CoA + H2O
fifth step in the leucine degradation pathway
-
-
?
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acetyl-coa c-acetyltransferase deficiency
Screening for defects of branched-chain amino acid metabolism.
Acidosis, Lactic
Hypertrophic cardiomyopathy, cataract, developmental delay, lactic acidosis: a novel subtype of 3-methylglutaconic aciduria.
Barth Syndrome
Association of 3-methylglutaconic aciduria with sensori-neural deafness, encephalopathy, and Leigh-like syndrome (MEGDEL association) in four patients with a disorder of the oxidative phosphorylation.
Cardiomyopathy, Dilated
3-Methylglutaconyl-Coenzyme-A Hydratase Deficiency and the Development of Dilated Cardiomyopathy.
Cardiomyopathy, Hypertrophic
Hypertrophic cardiomyopathy, cataract, developmental delay, lactic acidosis: a novel subtype of 3-methylglutaconic aciduria.
Cataract
Hypertrophic cardiomyopathy, cataract, developmental delay, lactic acidosis: a novel subtype of 3-methylglutaconic aciduria.
Dehydration
Metabolic reconstructions identify plant 3-methylglutaconyl-CoA hydratase that is crucial for branched-chain amino acid catabolism in mitochondria.
Heart Failure
3-Methylglutaconyl-Coenzyme-A Hydratase Deficiency and the Development of Dilated Cardiomyopathy.
hydroxymethylglutaryl-coa lyase deficiency
3-Methylglutaconyl-CoA hydratase, 3-methylcrotonyl-CoA carboxylase and 3-hydroxy-3-methylglutaryl-CoA lyase deficiencies: a coupled enzyme assay useful for their detection.
hydroxymethylglutaryl-coa lyase deficiency
Screening for defects of branched-chain amino acid metabolism.
isovaleryl-coa dehydrogenase deficiency
Screening for defects of branched-chain amino acid metabolism.
Metabolic Diseases
3-Methylglutaconic aciduria type I: clinical heterogeneity as a neurometabolic disease.
methylcrotonoyl-coa carboxylase deficiency
Screening for defects of branched-chain amino acid metabolism.
methylglutaconyl-coa hydratase deficiency
3-Methylglutaconic aciduria type I: clinical heterogeneity as a neurometabolic disease.
methylglutaconyl-coa hydratase deficiency
3-Methylglutaconic aciduria-lessons from 50 genes and 977 patients.
methylglutaconyl-coa hydratase deficiency
3-Methylglutaconic and 3-methylglutaric aciduria in a patient with suspected 3-methylglutaconyl-CoA hydratase deficiency.
methylglutaconyl-coa hydratase deficiency
3-Methylglutaconyl-CoA hydratase deficiency: a new patient with speech retardation as the leading sign.
methylglutaconyl-coa hydratase deficiency
3-Methylglutaconyl-Coenzyme-A Hydratase Deficiency and the Development of Dilated Cardiomyopathy.
methylglutaconyl-coa hydratase deficiency
Leucine Loading Test is Only Discriminative for 3-Methylglutaconic Aciduria Due to AUH Defect.
methylglutaconyl-coa hydratase deficiency
Screening for defects of branched-chain amino acid metabolism.
methylglutaconyl-coa hydratase deficiency
[3-Methylglutaconyl-CoA hydratase deficiency]
Starvation
Metabolic reconstructions identify plant 3-methylglutaconyl-CoA hydratase that is crucial for branched-chain amino acid catabolism in mitochondria.
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0.0083
(E)-3-methylglutaconyl-CoA
-
0.0024 - 0.04
(E)-glutaconyl-CoA
2.25
(R,S)-3-hydroxy-3-methylglutaryl-CoA
-
0.03 - 0.0531
(S)-3-hydroxy-3-methylglutaryl-CoA
0.05
(S)-3-hydroxyglutaryl-CoA
dehydration
0.0069 - 0.0094
(S)-3-hydroxymethylglutaryl-CoA
55.2
3-hydroxybutyryl-CoA
-
0.347
3-methylcrotonyl-CoA
-
0.01
3-methylglutaconyl-CoA
-
0.0291 - 0.08
trans-3-methylglutaconyl-CoA
additional information
additional information
-
0.0024
(E)-glutaconyl-CoA
-
0.04
(E)-glutaconyl-CoA
hydration
0.03
(S)-3-hydroxy-3-methylglutaryl-CoA
-
0.0531
(S)-3-hydroxy-3-methylglutaryl-CoA
pH 8.0, 30°C
0.0069
(S)-3-hydroxymethylglutaryl-CoA
-
fibroblast enzyme
0.0094
(S)-3-hydroxymethylglutaryl-CoA
-
lymphocyte enzyme
0.0291
trans-3-methylglutaconyl-CoA
-
pH 7.9, 25°C, wild-type enzyme
0.043
trans-3-methylglutaconyl-CoA
-
pH 7.9, 25°C, mutant enzyme E138Q
0.08
trans-3-methylglutaconyl-CoA
-
pH 7.9, 25°C, mutant enzyme E138D
additional information
additional information
addition of water to the (E)-isomer of 3-methylglutaconyl-CoA of the leucine degradative pathway, intermediate is (S)-3-hydroxy-3-methylglutaryl-CoA
-
additional information
additional information
-
addition of water to the (E)-isomer of 3-methylglutaconyl-CoA of the leucine degradative pathway, intermediate is (S)-3-hydroxy-3-methylglutaryl-CoA
-
additional information
additional information
kinetic constants determined by HPLC and mass spectrometry, assay conditions indicated
-
additional information
additional information
-
kinetic constants determined by HPLC and mass spectrometry, assay conditions indicated
-
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additional information
assay in presence of 0.5 ml 50 mM potassium phosphate pH 7.0 and 0.1 mM glutaconyl-CoA, kinetic constants determined by HPLC, 3-methylated CoA-thiol esters identified as the real substrates as expected from the pathway of isovalerate oxidation, specificity constants of other substrates as glutaconyl-CoA and 3-hydroxyglutaryl-CoA are 22 and 7 times lower, no activities measured for crotonyl-CoA
additional information
-
assay in presence of 0.5 ml 50 mM potassium phosphate pH 7.0 and 0.1 mM glutaconyl-CoA, kinetic constants determined by HPLC, 3-methylated CoA-thiol esters identified as the real substrates as expected from the pathway of isovalerate oxidation, specificity constants of other substrates as glutaconyl-CoA and 3-hydroxyglutaryl-CoA are 22 and 7 times lower, no activities measured for crotonyl-CoA
additional information
-
highly sensitive assay method, that allows reliable determination of enzyme activity in crude extract
additional information
activity of 3-methylglutaconyl-CoA hydratase measured in fibroblasts within normal limits in all patients, urinary organic acid analysis determined by HPLC or mass spectrometry, increase of 3-methylglutaconic acid in all cases, and 3-methylglutaric acid in four out five patients, three novel subtypes of the diseease 3-methylglutaconic aciduria not coupled to impaired activity of 3-methylglutaconyl-CoA hydratase
additional information
-
activity of 3-methylglutaconyl-CoA hydratase measured in fibroblasts within normal limits in all patients, urinary organic acid analysis determined by HPLC or mass spectrometry, increase of 3-methylglutaconic acid in all cases, and 3-methylglutaric acid in four out five patients, three novel subtypes of the diseease 3-methylglutaconic aciduria not coupled to impaired activity of 3-methylglutaconyl-CoA hydratase
additional information
direct nonisotopic activity assay in human skin fibroblast homogenates developed, products analyzed by HPLC, AUH-decifiency by mutations reveals 9% of the wild-type enzyme activity and increased levels of 3-methylglutaric acid and 3-hydroxyisovaleric acid
additional information
-
direct nonisotopic activity assay in human skin fibroblast homogenates developed, products analyzed by HPLC, AUH-decifiency by mutations reveals 9% of the wild-type enzyme activity and increased levels of 3-methylglutaric acid and 3-hydroxyisovaleric acid
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Messner, B.; Eggerer, H.; Cornforth, J.W.; Mallaby, R.
Substrate stereochemistry of the hydroxymethylglutaryl-CoA lyase and methylglutaconyl-CoA hydratase reactions
Eur. J. Biochem.
53
255-264
1975
Ovis aries
-
brenda
Hilz, H.; Knappe, J.; Ringelmann, E.; Lynen, F.
Methylglutaconase, eine neue Hydratase, die am Stoffwechsel verzweigter Carbonsaeuren beteiligt ist
Biochem. Z.
329
476-489
1958
Bos taurus
-
brenda
Hermans-Lokkerbol, A.; van der Heijden, R.; Verpoorte, R.
Isocratic high-performance liquid chromatography of coenzyme A esters involved in the metabolism of 3S-hydroxy-3-methylglutaryl-coenzyme A. Detection of related enzyme activities in Catharanthus roseus plant cell culture
J. Chromatogr. A
752
123-130
1996
Catharanthus roseus
-
brenda
Gibson, K.M.
Assay of 3-methylglutaconyl-CoA hydratase
Methods Enzymol.
166
214-218
1988
Homo sapiens
brenda
Gibson, K.M.
3-Methylglutaconyl-coenzyme-A hydratase deficiency: a new case
J. Inherit. Metab. Dis.
15
363-366
1992
Homo sapiens
brenda
Narisawa, K.; Gibson, K.M.; Sweetman, L.; Nyhan, W.L.
3-Methylglutaconyl-CoA hydratase, 3-methylcrotonyl-CoA carboxylase and 3-hydroxy-3-methylglutaryl-CoA lyase deficiencies: a coupled enzyme assay useful for their detection
Clin. Chim. Acta
184
57-64
1989
Homo sapiens
brenda
Van der Heijden, R.; de Boer-Hlupa, V.; Verpoorte, R.; Duine, J.A.
Enzymes involved in the metabolism of 3-hydroxy-3-methylglutaryl-coenzyme A in Catharanthus roseus
Plant Cell
38
345-349
1994
Catharanthus roseus
-
brenda
Wong, B.J.; Gerlt, J.A.
Evolution of function in the crotonase superfamily: (3S)-methylglutaconyl-CoA hydratase from Pseudomonas putida
Biochemistry
43
4646-4654
2004
Pseudomonas putida, Pseudomonas putida PA2011
brenda
Loupatty, F.J.; Ruiter, J.P.N.; Ijlst, L.; Duran, M.; Wanders, R.J.A.
Direct nonisotopic assay of 3-methylglutaconyl-CoA hydratase in cultured human skin fibroblasts to specifically identify patients with 3-methylglutaconic aciduria type I
Clin. Chem.
50
1447-1450
2004
Homo sapiens
brenda
Mack, M.; Liesert, M.; Zschocke, J.; Peters, V.; Linder, D.; Buckel, W.
3-Methylglutaconyl-CoA hydratase from Acinetobacter sp
Arch. Microbiol.
185
297-306
2006
Acinetobacter sp. (Q3HW12), Acinetobacter sp., Acinetobacter sp. DSM 11652 (Q3HW12)
brenda
Mack, M.; Schniegler-Mattox, U.; Peters, V.; Hoffmann, G.F.; Liesert, M.; Buckel, W.; Zschocke, J.
Biochemical characterization of human 3-methylglutaconyl-CoA hydratase and its role in leucine metabolism
FEBS J.
273
2012-2022
2006
Homo sapiens (Q13825), Homo sapiens
brenda
Di Rosa, G.; Deodato, F.; Loupatty, F.J.; Rizzo, C.; Carrozzo, R.; Santorelli, F.M.; Boenzi, S.; DAmico, A.; Tozzi, G.; Bertini, E.; Maiorana, A.; Wanders, R.J.; Dionisi-Vici, C.
Hypertrophic cardiomyopathy, cataract, developmental delay, lactic acidosis: a novel subtype of 3-methylglutaconic aciduria
J. Inherit. Metab. Dis.
29
546-550
2006
Homo sapiens (Q13825), Homo sapiens
brenda
Kurimoto, K.; Kuwasako, K.; Sandercock, A.; Unzai, S.; Robinson, C.; Muto, Y.; Yokoyama, S.
AU-rich RNA-binding induces changes in the quaternary structure of AUH
Proteins
75
360-372
2009
Homo sapiens (Q13825), Homo sapiens
brenda
Mercimek-Mahmutoglu, S.; Tucker, T.; Casey, B.
Phenotypic heterogeneity in two siblings with 3-methylglutaconic aciduria type I caused by a novel intragenic deletion
Mol. Genet. Metab.
104
410-413
2011
Homo sapiens
brenda
Su, B.; Ryan, R.O.
Metabolic biology of 3-methylglutaconic acid-uria: a new perspective
J. Inherit. Metab. Dis.
37
359-368
2014
Homo sapiens (Q13825)
brenda
Bock, T.; Reichelt, J.; Mueller, R.; Blankenfeldt, W.
The Structure of LiuC, a 3-hydroxy-3-methylglutaconyl CoA dehydratase involved in isovaleryl-CoA biosynthesis in Myxococcus xanthus, reveals insights into specificity and catalysis
ChemBioChem
17
1658-1664
2016
Myxococcus xanthus (Q1D5Y4), Myxococcus xanthus DK 1622 (Q1D5Y4)
brenda
de Lima Stein, M.L.; Icimoto, M.Y.; de Castro Levatti, E.V.; Oliveira, V.; Straus, A.H.; Schenkman, S.
Characterization and role of the 3-methylglutaconyl coenzyme A hidratase in Trypanosoma brucei
Mol. Biochem. Parasitol.
214
36-46
2017
Trypanosoma brucei, Trypanosoma brucei Lister 427
brenda
Latimer, S.; Li, Y.; Nguyen, T.T.H.; Soubeyrand, E.; Fatihi, A.; Elowsky, C.G.; Block, A.; Pichersky, E.; Basset, G.J.
Metabolic reconstructions identify plant 3-methylglutaconyl-CoA hydratase that is crucial for branched-chain amino acid catabolism in mitochondria
Plant J.
95
358-370
2018
Arabidopsis thaliana (F4JML5)
brenda