1.1.1.178: 3-hydroxy-2-methylbutyryl-CoA dehydrogenase
This is an abbreviated version!
For detailed information about 3-hydroxy-2-methylbutyryl-CoA dehydrogenase, go to the full flat file.
Word Map on EC 1.1.1.178
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1.1.1.178
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l-3-hydroxyacyl-coa
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2-methyl-3-hydroxybutyric
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hyperinsulinism
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tiglylglycine
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hydroxyacyl-coas
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choreoathetosis
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medicine
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lchad
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kcnj11
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2-methylacetoacetate
- 1.1.1.178
- l-3-hydroxyacyl-coa
-
2-methyl-3-hydroxybutyric
- hyperinsulinism
-
tiglylglycine
-
hydroxyacyl-coas
-
choreoathetosis
- medicine
- lchad
-
kcnj11
- 2-methylacetoacetate
Reaction
Synonyms
17beta-HSD10, 17beta-hydroxysteroid dehydrogenase type 10, 2-methyl-3-hydroxy-butyryl CoA dehydrogenase, 2-methyl-3-hydroxybutyryl coenzyme A dehydrogenase, 2-methyl-3-hydroxybutyryl-CoA dehydrogenase, 3-hydroxy-2-methylbutyryl-CoA dehydrogenase, 3-hydroxyacyl-CoA dehydrogenase type II, dehydrogenase, 2-methyl-3-hydroxybutyryl coenzyme A, HADII, HSD10, MHBD, SCHAD, short chain 3-hydroxyacyl-CoA dehydrogenase, short-chain L-3-hydroxy-2-methylacyl-CoA dehydrogenase, type II 3-hydroxyacyl-CoA dehydrogenase
ECTree
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General Information
General Information on EC 1.1.1.178 - 3-hydroxy-2-methylbutyryl-CoA dehydrogenase
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physiological function
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enzyme knock-down by antisense-oligonucleotides results in a 40% reduction in pyruvate turnover compared to the uninjected or control injected tissue. The morphology of mitochondria in animal cap explants is changed after HSD10 knock-down. Mitochondria show severe reduction of cristae and a generally irregular shape
physiological function
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generation of conditional Hsd17b10 knock-out mouse lines by elimination of Hsd17b10 in endothelial cells and cells of the immune system. These mice are viable and fertile, but have defects in spleen and vasculature. The animals die rapidly around week 25. Conditional knock-out lines with elimination of the Hsd17b10 gene in noradrenergic neurons are viable and fertile but die around week 26. In the loci coerulei of HSD10 deficient mice in noradrenergic neurons, almost 30% of the mitochondria show depletion of cristae and appeared empty, more than 50% of the mitochondria are loosely packed and have swollen cristae, while normal morphology is only found in 20%