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3-hydroxyacyl-CoA dehydrogenase type-2 Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Enoyl-CoA hydratase, mitochondrial Trans-2-enoyl-CoA reductase, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial 3-hydroxyacyl-CoA dehydrogenase type-2 Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Enoyl-CoA hydratase, mitochondrial Trans-2-enoyl-CoA reductase, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial 3-hydroxyacyl-CoA dehydrogenase type-2 Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Enoyl-CoA hydratase, mitochondrial Trans-2-enoyl-CoA reductase, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Enoyl-CoA hydratase, mitochondrial Trans-2-enoyl-CoA reductase, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Enoyl-CoA hydratase, mitochondrial Trans-2-enoyl-CoA reductase, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Enoyl-CoA hydratase, mitochondrial Trans-2-enoyl-CoA reductase, mitochondrial Palmitoyl-protein thioesterase 1 3- Hydroxyhexanoyl- CoA NAD 3-oxohexanoyl-CoA NADH (2E)-Hexenoyl-CoA H2O NADPH Hexanoyl-CoA NADP Acetyl-CoA Butyryl-CoA CoA Acetyl-CoA 3-oxooctanoyl-CoA CoA 3- hydroxyoctanoyl- CoA NAD NADH (2E)-Octenoyl-CoA H2O NADPH Octanoyl-CoA NADP Acetyl-CoA 3-oxodecanoyl-CoA CoA 3- Hydroxydecanoyl- CoA NAD NADH (2E)-Decenoyl-CoA H2O NADPH Decanoyl-CoA NADP Acetyl-CoA 3- oxododecanoyl- CoA CoA 3- Hydroxydodecanoyl- CoA NAD NADH (2E)- Dodecenoyl-CoA H2O NADPH Lauroyl-CoA NADP Acetyl-CoA 3- oxotetradecanoyl- CoA CoA 3- Hydroxytetradecanoyl- CoA NAD NADH (2E)- Tetradecenoyl- CoA H2O NADPH Tetradecanoyl-CoA NADP Acetyl-CoA 3- oxohexadecanoyl- CoA CoA 3- Hydroxyhexadecanoyl- CoA NAD NADH (2E)- Hexadecenoyl- CoA H2O NADPH Palmitoyl-CoA NADP H2O Palmitic acid CoA Cytosol Mitochondrial Space Mitochondrion Hypoglycemia, lethargy, irritability, hypertrophic cardiomyopathy and other heart conditions
Mitochondria HSD17B10 HADHA HADHB ECHS1 MECR ACAA2 ACAA2 HSD17B10 HADHA HADHB ECHS1 MECR ACAA2 HSD17B10 HADHA HADHB ECHS1 MECR ACAA2 HADHA HADHB ECHS1 MECR ACAA2 HADHA HADHB ECHS1 MECR ACAA2 HADHA HADHB ECHS1 MECR PPT1 3- Hydroxyhexanoyl- CoA NAD 3-oxohexanoyl-CoA NADH (2E)-Hexenoyl-CoA Water NADPH Hexanoyl-CoA NADP Acetyl-CoA Butyryl-CoA Coenzyme A Acetyl-CoA 3-oxooctanoyl-CoA Coenzyme A 3- hydroxyoctanoyl- CoA NAD NADH (2E)-Octenoyl-CoA Water NADPH Octanoyl-CoA NADP Acetyl-CoA 3-oxodecanoyl-CoA Coenzyme A 3- Hydroxydecanoyl- CoA NAD NADH (2E)-Decenoyl-CoA Water NADPH Decanoyl-CoA NADP Acetyl-CoA 3- oxododecanoyl- CoA Coenzyme A 3- Hydroxydodecanoyl- CoA NAD NADH (2E)- Dodecenoyl-CoA Water NADPH Lauroyl-CoA NADP Acetyl-CoA 3- oxotetradecanoyl- CoA Coenzyme A 3- Hydroxytetradecanoyl- CoA NAD NADH (2E)- Tetradecenoyl- CoA Water NADPH Tetradecanoyl-CoA NADP Acetyl-CoA 3- oxohexadecanoyl- CoA Coenzyme A 3- Hydroxyhexadecanoyl- CoA NAD NADH (2E)- Hexadecenoyl- CoA Water NADPH Palmitoyl-CoA NADP Water Palmitic acid Coenzyme A
HSD17B10 HADHA HADHB ECHS1 MECR ACAA2 ACAA2 HSD17B10 HADHA HADHB ECHS1 MECR ACAA2 HSD17B10 HADHA HADHB ECHS1 MECR ACAA2 HADHA HADHB ECHS1 MECR ACAA2 HADHA HADHB ECHS1 MECR ACAA2 HADHA HADHB ECHS1 MECR PPT1 S-HHxCA NAD 3OHOCoA NADH T2HCoA H2O NADPH COA6:0 NADP Ac-CoA Btyl-CA CoA Ac-CoA 3OOACoA CoA S-HOCoA NAD NADH 2OCoA H2O NADPH COA8:0 NADP Ac-CoA 3ODCCoA CoA SHD-CoA NAD NADH 2DCoA H2O NADPH Decanoy NADP Ac-CoA 3ODDCoA CoA S3HDCoA NAD NADH DDC-CoA H2O NADPH COA12:0 NADP Ac-CoA 3OTDCoA CoA S3HTCoA NAD NADH 2TCoA H2O NADPH COA14:0 NADP Ac-CoA 3OHDCoA CoA BHC NAD NADH (E)-C16 H2O NADPH COA16:0 NADP H2O 16:0 CoA Cytosol Mitochondrial Space Mitochondrion Hypoglycemia, lethargy, irritability, hypertrophic cardiomyopathy and other heart conditions
Mitochondria HSD17B10 HADHA HADHB ECHS1 MECR ACAA2 ACAA2 HSD17B10 HADHA HADHB ECHS1 MECR ACAA2 HSD17B10 HADHA HADHB ECHS1 MECR ACAA2 HADHA HADHB ECHS1 MECR ACAA2 HADHA HADHB ECHS1 MECR ACAA2 HADHA HADHB ECHS1 MECR PPT1 S-HHxCA NAD 3OHOCoA NADH T2HCoA H2O NADPH COA6:0 NADP Ac-CoA Btyl-CA CoA Ac-CoA 3OOACoA CoA S-HOCoA NAD NADH 2OCoA H2O NADPH COA8:0 NADP Ac-CoA 3ODCCoA CoA SHD-CoA NAD NADH 2DCoA H2O NADPH Decanoy NADP Ac-CoA 3ODDCoA CoA S3HDCoA NAD NADH DDC-CoA H2O NADPH COA12:0 NADP Ac-CoA 3OTDCoA CoA S3HTCoA NAD NADH 2TCoA H2O NADPH COA14:0 NADP Ac-CoA 3OHDCoA CoA BHC NAD NADH (E)-C16 H2O NADPH COA16:0 NADP H2O 16:0 CoA