1.3.8.4: isovaleryl-CoA dehydrogenase
This is an abbreviated version!
For detailed information about isovaleryl-CoA dehydrogenase, go to the full flat file.
Word Map on EC 1.3.8.4
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1.3.8.4
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acidemia
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acyl-coa
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inborn
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3-methylcrotonyl-coa
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isovalerylglycine
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medium-chain
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lethargy
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flavoenzyme
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isobutyryl-coa
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acylcarnitine
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isovalerylcarnitine
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flavoprotein:ubiquinone
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glutaryl-coa
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2-methylbutyryl-coa
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3-hydroxyisovaleric
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octanoyl-coa
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sweaty
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diagnostics
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medicine
- 1.3.8.4
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acidemia
- acyl-coa
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inborn
- 3-methylcrotonyl-coa
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isovalerylglycine
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medium-chain
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lethargy
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flavoenzyme
- isobutyryl-coa
- acylcarnitine
- isovalerylcarnitine
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flavoprotein:ubiquinone
- glutaryl-coa
- 2-methylbutyryl-coa
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3-hydroxyisovaleric
- octanoyl-coa
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sweaty
- diagnostics
- medicine
Reaction
Synonyms
acyl-CoA dehydrogenase, EC 1.3.99.10, i3VD, iso(3)valeryl-CoA dehydrogenase, isovaleric-CoA dehydrogenase, isovaleroyl-coenzyme A dehydrogenase, isovaleryl-coenzyme A dehydrogenase, IVD, IVDH, LiuA, Pden_3633, SBCAD, short/branched chain acyl-CoA dehydrogenase
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General Information
General Information on EC 1.3.8.4 - isovaleryl-CoA dehydrogenase
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evolution
malfunction
metabolism
physiological function
additional information
evolution
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isovaleryl-CoA dehydrogenase (and its corresponding gene) is widely distributed in mammals, plants, and bacteria. This enzyme belongs to the acyl-CoA dehydrogenase family, which are responsible for hydrogen transfer in flavoproteins
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deficiency in isovaleryl-CoA dehydrogenase causes isovaleric acidemia, a rare inherited metabolic disease
malfunction
during sugar starvation arising from the exposure of wild-type plants to darkness, autophagic transport of chloroplast stroma, which contains most of the proteins in a leaf, into the vacuolar lumen is induced within 2 days. During this time, the level of soluble proteins, primarily Rubisco (ribulose-1,5-bisphosphate carboxylase/oxygenase), decreases and the amount of free amino acid increases. In dark-treated autophagy-defective (atg) mutants, the decrease of soluble proteins is suppressed, which results in the compromised release of basic amino acids, branched-chain amino acids (BCAAs) and aromatic amino acids. The impairment of BCAA catabolic pathways in the knockout mutants of the electron transfer flavoprotein (ETF)/ETF:ubiquinone oxidoreductase (etfqo) complex and the electron donor protein isovaleryl-CoA dehydrogenase (ivdh) cause a reduced tolerance to dark treatment similar to that in the atg mutants. The enhanced accumulation of BCAAs in the ivdh and etfqo mutants during the dark treatment is reduced by additional autophagy deficiency. These results indicate that vacuolar protein degradation via autophagy serves as an adaptive response to disrupted photosynthesis by providing substrates to amino acid catabolic pathways, including BCAA catabolism mediated by IVDH and ETFQO. Knockout mutants atg10-1, atg5-1, and atg2-1, phenotypes, overview
malfunction
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during sugar starvation arising from the exposure of wild-type plants to darkness, autophagic transport of chloroplast stroma, which contains most of the proteins in a leaf, into the vacuolar lumen is induced within 2 days. During this time, the level of soluble proteins, primarily Rubisco (ribulose-1,5-bisphosphate carboxylase/oxygenase), decreases and the amount of free amino acid increases. In dark-treated autophagy-defective (atg) mutants, the decrease of soluble proteins is suppressed, which results in the compromised release of basic amino acids, branched-chain amino acids (BCAAs) and aromatic amino acids. The impairment of BCAA catabolic pathways in the knockout mutants of the electron transfer flavoprotein (ETF)/ETF:ubiquinone oxidoreductase (etfqo) complex and the electron donor protein isovaleryl-CoA dehydrogenase (ivdh) cause a reduced tolerance to dark treatment similar to that in the atg mutants. The enhanced accumulation of BCAAs in the ivdh and etfqo mutants during the dark treatment is reduced by additional autophagy deficiency. These results indicate that vacuolar protein degradation via autophagy serves as an adaptive response to disrupted photosynthesis by providing substrates to amino acid catabolic pathways, including BCAA catabolism mediated by IVDH and ETFQO. Knockout mutants atg10-1, atg5-1, and atg2-1, phenotypes, overview
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isovaleryl-CoA dehydrogenase is an important enzyme in branched chain amino acid metabolism. The pathway of leucine to mevalonate in halophilic archaea involves efficient incorporation of leucine into isoprenoidal lipid with the requirement of isovaleryl-CoA dehydrogenase in Halobacterium salinarum, overview
metabolism
BCAA transaminase 2 (BCAT2) and the branched-chain alpha2-oxo acid dehydrogenase complex subunit E1A1 (BCKDH E1A1) are involved in BCAA catabolism by providing substrates to enzyme IVDH. During sugar starvation arising from the exposure of wild-type plants to darkness, autophagic transport of chloroplast stroma, which contains most of the proteins in a leaf, into the vacuolar lumen is induced within 2 days. During this time, the level of soluble proteins, primarily Rubisco (ribulose-1,5-bisphosphate carboxylase/oxygenase), decreases and the amount of free amino acid increases. Vacuolar protein degradation via autophagy serves as an adaptive response to disrupted photosynthesis by providing substrates to amino acid catabolic pathways, including BCAA catabolism mediated by IVDH and ETFQO, involving the isovaleryl-CoA dehydrogenase (ivdh). Autophagy and amino acid catabolism are important in the plant response to sugar starvation. Relationship between autophagy and amino acid catabolism via the ETF/ETFQO system, overview
metabolism
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involvement of isovaleryl-CoA dehydrogenase in leucine conversion to isoprenoid lipid in halophilic archaea, involvement of the leucine-to-mevalonate pathway, especially isovaleryl-CoA dehydrogenase, in the production of 3-methylcrotonyl-CoA. Branched amino acids are metabolized to mevalonate in archaea in a manner similar to other organisms, metabolic pathway overview
metabolism
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isovaleryl-CoA dehydrogenase is an important enzyme in branched chain amino acid metabolism. The pathway of leucine to mevalonate in halophilic archaea involves efficient incorporation of leucine into isoprenoidal lipid with the requirement of isovaleryl-CoA dehydrogenase in Halobacterium salinarum, overview
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metabolism
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BCAA transaminase 2 (BCAT2) and the branched-chain alpha2-oxo acid dehydrogenase complex subunit E1A1 (BCKDH E1A1) are involved in BCAA catabolism by providing substrates to enzyme IVDH. During sugar starvation arising from the exposure of wild-type plants to darkness, autophagic transport of chloroplast stroma, which contains most of the proteins in a leaf, into the vacuolar lumen is induced within 2 days. During this time, the level of soluble proteins, primarily Rubisco (ribulose-1,5-bisphosphate carboxylase/oxygenase), decreases and the amount of free amino acid increases. Vacuolar protein degradation via autophagy serves as an adaptive response to disrupted photosynthesis by providing substrates to amino acid catabolic pathways, including BCAA catabolism mediated by IVDH and ETFQO, involving the isovaleryl-CoA dehydrogenase (ivdh). Autophagy and amino acid catabolism are important in the plant response to sugar starvation. Relationship between autophagy and amino acid catabolism via the ETF/ETFQO system, overview
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involvement of ivdh in phytol degradation during dark-Induced starvation
physiological function
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IVD catalyzes the stereospecific conversion of the diastereotopic methyl group of leucine to isoprenoidal lipids
physiological function
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IVD catalyzes the stereospecific conversion of the diastereotopic methyl group of leucine to isoprenoidal lipids
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physiological function
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involvement of ivdh in phytol degradation during dark-Induced starvation
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a G376V molecular defect of isovaleryl-CoA dehydrogenase, IVD, causes IVD deficiency which is responsible for the isovaleric acid-emanating skunk mutant odorous phenotype with prepupal lethality of the silkworm, molecular modelling, overview. The sku larvae begins emanating isovaleric acid odour from the first day after hatching, but does not show any signs of developmental abnormality until the onset of spinning. The mutants start spinning after the normal duration of the final instar, 6-8 days, but stop after a short time and develop a very thin cocoon. They eventually die without becoming pupae in about a week after spinning
additional information
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a G376V molecular defect of isovaleryl-CoA dehydrogenase, IVD, causes IVD deficiency which is responsible for the isovaleric acid-emanating skunk mutant odorous phenotype with prepupal lethality of the silkworm, molecular modelling, overview. The sku larvae begins emanating isovaleric acid odour from the first day after hatching, but does not show any signs of developmental abnormality until the onset of spinning. The mutants start spinning after the normal duration of the final instar, 6-8 days, but stop after a short time and develop a very thin cocoon. They eventually die without becoming pupae in about a week after spinning
additional information
impaired branched chain amino acid catabolic enzyme isovaleryl-CoA dehydrogenase causes a metabolic syndrome with increased seed homomethionine and isovaleroyloxypropyl-glucosinolate, along with reduced 3-benzoyloxypropyl-glucosinolate, a diverse set of metabolites is affected in the ivd1 mutants, complementary metabolite profiling analysis, overview
additional information
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impaired branched chain amino acid catabolic enzyme isovaleryl-CoA dehydrogenase causes a metabolic syndrome with increased seed homomethionine and isovaleroyloxypropyl-glucosinolate, along with reduced 3-benzoyloxypropyl-glucosinolate, a diverse set of metabolites is affected in the ivd1 mutants, complementary metabolite profiling analysis, overview
additional information
responses of Arabidopsis thaliana mutants deficient in the expression of isovaleryl-CoA dehydrogenase and 2-hydroxyglutarate dehydrogenase to extended darkness and other environmental stresses, phenotype, overview. Isovaleryl-CoA dehydrogenase is the more critical for alternative respiration. Both isovaleryl-CoA dehydrogenase and 2-hydroxyglutarate dehydrogenase act as electron donors to the ubiquinol pool via an electron-transfer flavoprotein/electron-transfer flavoprotein:ubiquinone oxidoreductase-mediated route
additional information
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responses of Arabidopsis thaliana mutants deficient in the expression of isovaleryl-CoA dehydrogenase and 2-hydroxyglutarate dehydrogenase to extended darkness and other environmental stresses, phenotype, overview. Isovaleryl-CoA dehydrogenase is the more critical for alternative respiration. Both isovaleryl-CoA dehydrogenase and 2-hydroxyglutarate dehydrogenase act as electron donors to the ubiquinol pool via an electron-transfer flavoprotein/electron-transfer flavoprotein:ubiquinone oxidoreductase-mediated route
additional information
residue E246 is the predicted active site catalytic residue of the enzyme
additional information
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the enzyme reactions stereochemical course is anti-elimination
additional information
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a G376V molecular defect of isovaleryl-CoA dehydrogenase, IVD, causes IVD deficiency which is responsible for the isovaleric acid-emanating skunk mutant odorous phenotype with prepupal lethality of the silkworm, molecular modelling, overview. The sku larvae begins emanating isovaleric acid odour from the first day after hatching, but does not show any signs of developmental abnormality until the onset of spinning. The mutants start spinning after the normal duration of the final instar, 6-8 days, but stop after a short time and develop a very thin cocoon. They eventually die without becoming pupae in about a week after spinning
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additional information
-
impaired branched chain amino acid catabolic enzyme isovaleryl-CoA dehydrogenase causes a metabolic syndrome with increased seed homomethionine and isovaleroyloxypropyl-glucosinolate, along with reduced 3-benzoyloxypropyl-glucosinolate, a diverse set of metabolites is affected in the ivd1 mutants, complementary metabolite profiling analysis, overview
-
additional information
-
responses of Arabidopsis thaliana mutants deficient in the expression of isovaleryl-CoA dehydrogenase and 2-hydroxyglutarate dehydrogenase to extended darkness and other environmental stresses, phenotype, overview. Isovaleryl-CoA dehydrogenase is the more critical for alternative respiration. Both isovaleryl-CoA dehydrogenase and 2-hydroxyglutarate dehydrogenase act as electron donors to the ubiquinol pool via an electron-transfer flavoprotein/electron-transfer flavoprotein:ubiquinone oxidoreductase-mediated route
-
additional information
-
residue E246 is the predicted active site catalytic residue of the enzyme
-
additional information
-
a G376V molecular defect of isovaleryl-CoA dehydrogenase, IVD, causes IVD deficiency which is responsible for the isovaleric acid-emanating skunk mutant odorous phenotype with prepupal lethality of the silkworm, molecular modelling, overview. The sku larvae begins emanating isovaleric acid odour from the first day after hatching, but does not show any signs of developmental abnormality until the onset of spinning. The mutants start spinning after the normal duration of the final instar, 6-8 days, but stop after a short time and develop a very thin cocoon. They eventually die without becoming pupae in about a week after spinning
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