4.2.1.18: methylglutaconyl-CoA hydratase
This is an abbreviated version!
For detailed information about methylglutaconyl-CoA hydratase, go to the full flat file.
Word Map on EC 4.2.1.18
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4.2.1.18
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acidurias
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3-methylglutaconic
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urine
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inborn
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3-methylglutaric
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speech
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3-methylcrotonyl-coa
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3-hydroxyisovaleric
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hypotonia
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enoyl-coas
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psychomotor
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isovaleryl-coa
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thiolase
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medicine
- 4.2.1.18
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acidurias
-
3-methylglutaconic
- urine
-
inborn
-
3-methylglutaric
-
speech
- 3-methylcrotonyl-coa
-
3-hydroxyisovaleric
-
hypotonia
- enoyl-coas
-
psychomotor
- isovaleryl-coa
-
thiolase
- medicine
Reaction
Synonyms
(3S)-methylglutaconyl-CoA hydratase, 3-methylglutaconyl CoA hydratase, 3-methylglutaconyl-CoA hydratase, 3-MG-CoA, 3-MGCoA-H, 3MGH, At4g16800, AU RNA binding protein/enoyl-Coenzyme A hydratase, AUH, HMG-CoA hydrolyase, hydratase, methylglutaconyl coenzyme A, LiuC, methylglutaconase, methylglutaconyl coenzyme A hydratase, MG-CoA hydratase, MGCH, Tb427.10.4000
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General Information
General Information on EC 4.2.1.18 - methylglutaconyl-CoA hydratase
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malfunction
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enzyme deficiency, due to homozygous deletion of exons 1-3 within the AUH gene, leads to 3-methylglutaconic aciduria type I with phenotypic heterogeneity, e.g. learning disability, attention deficit-hyperactivity and early onset subclinical leukoencephalopathy or severe expressive speech delay and delay in speech sound development with normal cognitive functions, overview
physiological function
at4g16800 knockout plants subjected to dark-induced carbon starvation, display accelerated senescence in their rosette leaves as compared with control plants, and a marked increase in the accumulation of free and total leucine, isoleucine and valine. The seeds of the at4g16800 mutant show a similar accumulation of free branched-chain amino acids
physiological function
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the depletion of 3-MGCoA-H by RNAi affects minimally the proliferation of both insect and mammalian bloodstream forms. An excess of leucine in the culture medium causes growth defects in cells depleted of 3-MGCoA-H, which can be reestablished by mevalonate. Procyclics depleted of the 3-MGCoA-H present reduced levels of synthesized steroids relative to cholesterol that is scavenged by the parasite
physiological function
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the depletion of 3-MGCoA-H by RNAi affects minimally the proliferation of both insect and mammalian bloodstream forms. An excess of leucine in the culture medium causes growth defects in cells depleted of 3-MGCoA-H, which can be reestablished by mevalonate. Procyclics depleted of the 3-MGCoA-H present reduced levels of synthesized steroids relative to cholesterol that is scavenged by the parasite
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