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Acyl-CoA synthetase short-chain family member 3, mitochondrial Enoyl-CoA hydratase, mitochondrial 3- hydroxyacyl- CoA dehydrogenase type-2 Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial Acetyl-CoA acetyltransferase, mitochondrial Enoyl-CoA hydratase, mitochondrial 3- hydroxyacyl- CoA dehydrogenase type-2 Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial Acetyl-CoA acetyltransferase, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial Short-chain specific acyl-CoA dehydrogenase, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Acetyl-CoA Acetyl-CoA Caproic acid Hexanoyl-CoA Caproic acid ATP CoA AMP PPi 3-Hydroxyhexanoyl-CoA (2E)-Hexenoyl-CoA H2O NAD 3-oxohexanoyl-CoA NADH H+ NAD NADH H+ Butyryl-CoA CoA 3-Hydroxybutyryl-CoA Crotonoyl-CoA H2O NAD Acetoacetyl-CoA NADH H+ NAD NADH H+ CoA CoA CoA electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein Citric Acid Cycle Citric Acid Cycle Fatty Acid Biosynthesis Fatty Acid Elongation in Mitochondria Mitochondrial Beta-Oxidation of Medium Chain Saturated Fatty Acids FAD FAD Mitochondrial Outer Membrane Mitochondrial Inner Membrane Mitochondrial Matrix Cytosol Symptoms of HADH deficiency include hypoglycemia, as well as vomiting, diarrhea and seizures.
Mitochondria ACSS3 ECHS1 HSD17B10 HADH ACAT1 ECHS1 HSD17B10 HADH ACAA2 ACAT1 ACAA2 ACADS ACADL Acetyl-CoA Acetyl-CoA Caproic acid Hexanoyl-CoA Caproic acid Adenosine triphosphate Coenzyme A Adenosine monophosphate Pyrophosphate 3- Hydroxyhexanoyl- CoA (2E)- Hexenoyl-CoA Water NAD 3- oxohexanoyl- CoA NADH Hydrogen Ion NAD NADH Hydrogen Ion Butyryl-CoA Coenzyme A 3- Hydroxybutyryl- CoA Crotonoyl-CoA Water NAD Acetoacetyl-CoA NADH Hydrogen Ion NAD NADH Hydrogen Ion Coenzyme A Coenzyme A Coenzyme A electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein Citric Acid Cycle Citric Acid Cycle Fatty Acid Biosynthesis Fatty Acid Elongation in Mitochondria Mitochondrial Beta-Oxidation of Medium Chain Saturated Fatty Acids
ACSS3 ECHS1 HSD17B10 HADH ACAT1 ECHS1 HSD17B10 HADH ACAA2 ACAT1 ACAA2 ACADS ACADL Ac-CoA Ac-CoA 6:0 COA6:0 6:0 ATP CoA AMP PPi S-HHxCA T2HCoA H2O NAD 3OHOCoA NADH H+ NAD NADH H+ Btyl-CA CoA 3HB-CoA CrtylCA H2O NAD ActaCoA NADH H+ NAD NADH H+ CoA CoA CoA ETF RETF ETF RETF PW000005 PW000005 PW000167 PW000022 PW000172 FAD FAD Mitochondrial Outer Membrane Mitochondrial Inner Membrane Mitochondrial Matrix Cytosol Symptoms of HADH deficiency include hypoglycemia, as well as vomiting, diarrhea and seizures.
Mitochondria ACSS3 ECHS1 HSD17B10 HADH ACAT1 ECHS1 HSD17B10 HADH ACAA2 ACAT1 ACAA2 ACADS ACADL Ac-CoA Ac-CoA 6:0 COA6:0 6:0 ATP CoA AMP Ppi S-HHxCA T2HCoA H2O NAD 3OHOCoA NADH H+ NAD NADH H+ Btyl-CA CoA 3HB-CoA CrtylCA H2O NAD ActaCoA NADH H+ NAD NADH H+ CoA CoA CoA ETF RETF ETF RETF PW000005 PW000005 PW000167 PW000022 PW000172