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Methylmalonyl- CoA epimerase, mitochondrial Methylmalonyl- CoA mutase, mitochondrial Malonyl-CoA decarboxylase, mitochondrial Acetyl-CoA carboxylase 1 Acetyl-CoA acetyltransferase, mitochondrial Methylmalonate- semialdehyde dehydrogenase [acylating], mitochondrial 4-aminobutyrate aminotransferase, mitochondrial Enoyl-CoA hydratase, mitochondrial 3- hydroxyisobutyryl- CoA hydrolase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Acetyl-coenzyme A synthetase 2-like, mitochondrial Acyl-CoA synthetase short-chain family member 3, mitochondrial Acyl-CoA synthetase short-chain family member 3, mitochondrial Acetyl-coenzyme A synthetase 2-like, mitochondrial Methylmalonate- semialdehyde dehydrogenase [acylating], mitochondrial Propionyl-CoA carboxylase alpha chain, mitochondrial Propionyl-CoA carboxylase beta chain, mitochondrial branched-chain alpha-keto dehydrogenase complex L-lactate dehydrogenase A-like 6B Acetyl-CoA Succinyl-CoA Propionic acid Hydroxypropionic acid Propionyl-CoA β-Alanine 2-Ketobutyric acid Citric acid Malonyl-CoA semialdehyde 3-Hydroxypropionyl-CoA S-Methylmalonyl-CoA R-Methylmalonyl-CoA L-Valine (S)-Methylmalonic acid semialdehyde Methylmalonic acid Malonyl-CoA CO2 ATP HCO3- Pi ADP Acetoacetyl-CoA CoA Malonic semialdehyde CoA NAD CO2 NADH Oxoglutaric acid L-Glutamic acid Acrylyl-CoA H2O H2O CoA AMP PPi Propinol adenylate ATP Pantetheine ATP Pantetheine AMP PPi AMP AMP H2O NAD CoA NADH HCO3- HCO3- ATP ADP H+ CoA NAD NADH 2-Hydroxybutyric acid NAD NADH H+ Adenosylcobalamin Pyridoxal 5'-phosphate FAD electron- transfer flavoprotein Reduced electron- transfer flavoprotein Pyruvate Metabolism Citric Acid Cycle Valine, Leucine, and Isoleucine Degradation Pantothenate and CoA Biosynthesis Cysteine and Methionine Metabolism Glycine, Serine, and Threonine Metabolism Tricarboxylate Transport System Biotin Manganese Biotin FAD Thiamine pyrophosphate Mitochondria Mitochondrial Matrix Extracellular Space Delayed development, hypotonia, seizures, vomiting, diarrhea and cardiomyopathy.
Mitochondria MCEE MUT MLYCD ACACA ACAT1 ALDH6A1 ABAT ECHS1 HIBCH ACADM ACSS1 ACSS3 ACSS3 ACSS1 ALDH6A1 PCCA PCCB BCKDHA LDHAL6B Acetyl-CoA Succinyl-CoA Propionic acid Hydroxypropionic acid Propionyl-CoA β-Alanine 2-Ketobutyric acid Citric acid Malonyl-CoA semialdehyde 3- Hydroxypropionyl- CoA S- Methylmalonyl- CoA R- Methylmalonyl- CoA L-Valine (S)- Methylmalonic acid semialdehyde Methylmalonic acid Malonyl-CoA Carbon dioxide Adenosine triphosphate Hydrogen carbonate Phosphoric acid Adenosine diphosphate Acetoacetyl-CoA Coenzyme A Malonic semialdehyde Coenzyme A NAD Carbon dioxide NADH Oxoglutaric acid L-Glutamic acid Acrylyl-CoA Water Water Coenzyme A Adenosine monophosphate Pyrophosphate Propinol adenylate Adenosine triphosphate Pantetheine Adenosine triphosphate Pantetheine Adenosine monophosphate Pyrophosphate Adenosine monophosphate Adenosine monophosphate Water NAD Coenzyme A NADH Hydrogen carbonate Hydrogen carbonate Adenosine triphosphate Adenosine diphosphate Hydrogen Coenzyme A NAD NADH 2- Hydroxybutyric acid NAD NADH Hydrogen Ion electron- transfer flavoprotein Reduced electron- transfer flavoprotein Pyruvate Metabolism Citric Acid Cycle Intestinal Microflora Valine, Leucine, and Isoleucine Degradation Pantothenate and CoA Biosynthesis Cysteine and Methionine Metabolism Glycine, Serine, and Threonine Metabolism Tricarboxylate Transport System
MCEE MUT MLYCD ACACA ACAT1 ALDH6A1 ABAT ECHS1 HIBCH ACADM ACSS1 ACSS3 ACSS3 ACSS1 ALDH6A1 PCCA PCCB BCKDHA LDHAL6B Ac-CoA Suc-CoA 3:0 3OH PropCoA b-Ala 2KBA CA MaCoAsa 3HP-CoA S-MmCoA R-MmCoA Val 22M3O Isosca MalyCoA CO2 ATP HCO3- Pi ADP ActaCoA CoA MalSah CoA NAD CO2 NADH AKG Glu AcryCoA H2O H2O CoA AMP PPi ProAdyl ATP Panethn ATP Panethn AMP PPi AMP AMP H2O NAD CoA NADH HCO3- HCO3- ATP ADP H+ CoA NAD NADH 2HBA NAD NADH H+ Adnscbn Pyr-5'P FAD ETF RETF PW000054 PW000005 PW000051 PW000032 C a M M G S a T Tr Tr S Biotin Mn2+ Biotin FAD ThiamPP Mitochondria Mitochondrial Matrix Extracellular Space Delayed development, hypotonia, seizures, vomiting, diarrhea and cardiomyopathy.
Mitochondria MCEE MUT MLYCD ACACA ACAT1 ALDH6A1 ABAT ECHS1 HIBCH ACADM ACSS1 ACSS3 ACSS3 ACSS1 ALDH6A1 PCCA PCCB BCKDHA LDHAL6B Ac-CoA Suc-CoA 3:0 3OH PropCoA b-Ala 2KBA CA MaCoAsa 3HP-CoA S-MmCoA R-MmCoA Val 22M3O Isosca MalyCoA CO2 ATP HCO3 PhosfrA ADP ActaCoA CoA MalSah CoA NAD CO2 NADH AKG Glu AcryCoA H2O H2O CoA AMP Ppi ProAdyl ATP Panethn ATP Panethn AMP Ppi AMP AMP H2O NAD CoA NADH HCO3 HCO3 ATP ADP H2 CoA NAD NADH 2HBA NAD NADH H+ ETF RETF PW000054 PW000005 Int Mic PW000051 PW000032 C a M M G S a T Tr Tr S