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(S)-beta-Aminoisobutyric acid Unknown 2- oxoisovalerate dehydrogenase subunit beta, mitochondrial 2- oxoisovalerate dehydrogenase subunit alpha, mitochondrial Dihydrolipoyl dehydrogenase, mitochondrial Hydroxymethylglutaryl- CoA lyase, mitochondrial 2- oxoisovalerate dehydrogenase subunit beta, mitochondrial 2- oxoisovalerate dehydrogenase subunit beta, mitochondrial Branched- chain-amino- acid aminotransferase, cytosolic Branched- chain-amino- acid aminotransferase, cytosolic Branched- chain-amino- acid aminotransferase, cytosolic 2- oxoisovalerate dehydrogenase Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial Isovaleryl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Enoyl-CoA hydratase, mitochondrial Methylcrotonoyl- CoA carboxylase subunit alpha, mitochondrial Methylcrotonoyl- CoA carboxylase beta chain, mitochondrial Dihydrolipoyl dehydrogenase, mitochondrial Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial 2- oxoisovalerate dehydrogenase 2- oxoisovalerate dehydrogenase Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial Short-chain specific acyl-CoA dehydrogenase, mitochondrial Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial Isobutyryl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Enoyl-CoA hydratase, mitochondrial 3- hydroxyisobutyryl- CoA hydrolase, mitochondrial 3- hydroxyisobutyrate dehydrogenase, mitochondrial Enoyl-CoA hydratase, mitochondrial 4-aminobutyrate aminotransferase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Short-chain specific acyl-CoA dehydrogenase, mitochondrial Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial Enoyl-CoA hydratase, mitochondrial 3- hydroxyacyl- CoA dehydrogenase type-2 3-ketoacyl-CoA thiolase, mitochondrial Methylmalonate- semialdehyde dehydrogenase [acylating], mitochondrial Propionyl-CoA carboxylase alpha chain, mitochondrial Propionyl-CoA carboxylase beta chain, mitochondrial Methylmalonyl- CoA epimerase, mitochondrial Methylmalonyl- CoA mutase, mitochondrial Aldehyde oxidase Aldehyde dehydrogenase, mitochondrial Methylglutaconyl- CoA hydratase, mitochondrial Hydroxymethylglutaryl- CoA synthase, mitochondrial Acetyl-CoA acetyltransferase, mitochondrial Succinyl- CoA:3- ketoacid coenzyme A transferase 1, mitochondrial 2- oxoisovalerate dehydrogenase subunit alpha, mitochondrial 2- oxoisovalerate dehydrogenase subunit alpha, mitochondrial Acetyl-CoA Propionyl-CoA Acetyl-CoA Succinyl-CoA 3-Hydroxy-3-methylglutaryl-CoA 3-Hydroxy-3-methylglutaryl-CoA 3-Methyl-1-hydroxybutyl-ThPP Lipoamide S-(3-Methylbutanoyl)-dihydrolipoamide-E Dihydrolipoamide NAD NADH H+ Acetoacetic acid 2-Methyl-1-hydroxypropyl-ThPP S-(2-Methylpropionyl)-dihydrolipoamide-E Lipoamide 2-Methyl-1-hydroxypropyl-ThPP S-(2-Methylbutanoyl)-dihydrolipoamide Thiamine pyrophosphate L-Valine Oxoglutaric acid α-Ketoisovaleric acid L-Glutamic acid L-Leucine Oxoglutaric acid Ketoleucine L-Glutamic acid L-Isoleucine Oxoglutaric acid 3-Methyl-2-oxovaleric acid L-Glutamic acid Thiamine pyrophosphate CO2 CoA Isovaleryl-CoA 3-Methylcrotonyl-CoA 3-Hydroxyisovaleryl-CoA H2O ATP HCO3- 3-Methylglutaconyl-CoA ADP Dihydrolipoamide NAD NADH H+ CoA Isobutyryl-CoA CO2 CO2 CoA 2-Methylbutyryl-CoA Methacrylyl-CoA H2O (S)-3-Hydroxyisobutyryl-CoA H2O (S)-3-Hydroxyisobutyric acid CoA NAD (S)-Methylmalonic acid semialdehyde NADH H+ Oxoglutaric acid L-Glutamic acid Tiglyl-CoA H2O 2-Methyl-3-hydroxybutyryl-CoA NAD 2-Methylacetoacetyl-CoA NADH CoA H2O NAD CoA NADH HCO3- ATP HCO3- S-Methylmalonyl-CoA ADP Pi R-Methylmalonyl-CoA H2O O2 Methylmalonic acid H2O2 NAD H2O NADH H2O Acetoacetyl-CoA H2O CoA CoA Succinyl-CoA Succinic acid Pyridoxal Pyridoxal FAD FAD FAD FAD FAD Biotin Adenosylcobalamin FAD Molybdopterin 2Fe-2S electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein Unknown Pyrimidine Metabolism Citric Acid Cycle Propanoate Metabolism Citric Acid Cycle Citric Acid Cycle Thiamine pyrophosphate Thiamine pyrophosphate FAD Thiamine pyrophosphate Pyridoxal Pyridoxal 5'-phosphate Lipoyl-GMP FAD FAD Biotin FAD Lipoyl-GMP Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Lipoyl-GMP FAD FAD Pyridoxal 5'-phosphate Mitochondria Cytosol Pancreas Symptoms include cardiomyopathy, dehydration, hypotonia, lactic acidosis, and pancreatitis. Treatment includes a low-fat, low-protein, high-carbohydrate diet. Muscle Heart
Mitochondria (S)-beta- Aminoisobutyric acid Unknown BCKDHB BCKDHA DLD HMGCL BCKDHB BCKDHB BCAT1 BCAT1 BCAT1 BCKDHA BCKDHB DBT IVD ACADM ECHS1 MCCC1 MCCC2 DLD DBT BCKDHA BCKDHB BCKDHA BCKDHB DBT ACADS ACADSB ACAD8 ACADM ECHS1 HIBCH HIBADH ECHS1 ABAT ACADM ACADS ACADSB ECHS1 HSD17B10 ACAA2 ALDH6A1 PCCA PCCB MCEE MUT AOX1 ALDH2 AUH HMGCS2 ACAT1 OXCT1 BCKDHA BCKDHA Acetyl-CoA Propionyl-CoA Acetyl-CoA Succinyl-CoA 3-Hydroxy-3- methylglutaryl- CoA 3-Hydroxy-3- methylglutaryl- CoA 3-Methyl-1- hydroxybutyl- ThPP Lipoamide S-(3- Methylbutanoyl)- dihydrolipoamide- E Dihydrolipoamide NAD NADH Hydrogen Ion Acetoacetic acid 2-Methyl-1- hydroxypropyl- ThPP S-(2- Methylpropionyl)- dihydrolipoamide- E Lipoamide 2-Methyl-1- hydroxypropyl- ThPP S-(2- Methylbutanoyl)- dihydrolipoamide Thiamine pyrophosphate L-Valine Oxoglutaric acid α- Ketoisovaleric acid L-Glutamic acid L-Leucine Oxoglutaric acid Ketoleucine L-Glutamic acid L-Isoleucine Oxoglutaric acid 3-Methyl-2- oxovaleric acid L-Glutamic acid Thiamine pyrophosphate Carbon dioxide Coenzyme A Isovaleryl-CoA 3- Methylcrotonyl- CoA 3- Hydroxyisovaleryl- CoA Water Adenosine triphosphate Hydrogen carbonate 3- Methylglutaconyl- CoA Adenosine diphosphate Dihydrolipoamide NAD NADH Hydrogen Ion Coenzyme A Isobutyryl-CoA Carbon dioxide Carbon dioxide Coenzyme A 2- Methylbutyryl- CoA Methacrylyl-CoA Water (S)-3- Hydroxyisobutyryl- CoA Water (S)-3- Hydroxyisobutyric acid Coenzyme A NAD (S)- Methylmalonic acid semialdehyde NADH Hydrogen Ion Oxoglutaric acid L-Glutamic acid Tiglyl-CoA Water 2-Methyl-3- hydroxybutyryl- CoA NAD 2- Methylacetoacetyl- CoA NADH Coenzyme A Water NAD Coenzyme A NADH Hydrogen carbonate Adenosine triphosphate Hydrogen carbonate S- Methylmalonyl- CoA Adenosine diphosphate Phosphate R- Methylmalonyl- CoA Water Oxygen Methylmalonic acid Hydrogen peroxide NAD Water NADH Water Acetoacetyl-CoA Water Coenzyme A Coenzyme A Succinyl-CoA Succinic acid electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein Unknown Pyrimidine Metabolism Citric Acid Cycle Propanoate Metabolism Citric Acid Cycle Citric Acid Cycle
L2MBA BCKDHB BCKDHA DLD HMGCL BCKDHB BCKDHB BCAT1 BCAT1 BCAT1 BCKDHA BCKDHB DBT IVD ACADM ECHS1 MCCC1 MCCC2 DLD DBT BCKDHA BCKDHB BCKDHA BCKDHB DBT ACADS ACADSB ACAD8 ACADM ECHS1 HIBCH HIBADH ECHS1 ABAT ACADM ACADS ACADSB ECHS1 HSD17B10 ACAA2 ALDH6A1 PCCA PCCB MCEE MUT AOX1 ALDH2 AUH HMGCS2 ACAT1 OXCT1 BCKDHA BCKDHA Ac-CoA PropCoA Ac-CoA Suc-CoA HMG-CoA HMG-CoA 3MhThPP Lipoamd S3MdipE DHLipoa NAD NADH H+ LIN 2M1ThPP S2MDE Lipoamd 2M1ThPP S2MD ThiamPP Val AKG Ketoval Glu Leu AKG Ketoleu Glu Ile AKG 3M2Oxva Glu ThiamPP CO2 CoA IsovCoA 3McroCA HIV-CoA H2O ATP HCO3- 3MCoA ADP DHLipoa NAD NADH H+ CoA IsoBCoA CO2 CO2 CoA 2IbuCoA McryCoA H2O 3HIsoCA H2O 3-HIBA CoA NAD 22M3O NADH H+ AKG Glu Tig-CoA H2O 2M3HC NAD 2Ma-CoA NADH CoA H2O NAD CoA NADH HCO3- ATP HCO3- S-MmCoA ADP Pi R-MmCoA H2O O2 Isosca H2O2 NAD H2O NADH H2O ActaCoA H2O CoA CoA Suc-CoA Succini Pyridox Pyridox FAD FAD FAD FAD FAD Biotin Adnscbn FAD mlyBdpn 2Fe2S ETF RETF ETF RETF ETF RETF PW000160 PW000005 PW000149 PW000005 PW000005 ThiamPP ThiamPP FAD ThiamPP Pyridox Pyr-5'P L-GMP FAD FAD Biotin FAD L-GMP Pyr-5'P Pyr-5'P L-GMP FAD FAD Pyr-5'P Mitochondria Cytosol Pancreas Symptoms include cardiomyopathy, dehydration, hypotonia, lactic acidosis, and pancreatitis. Treatment includes a low-fat, low-protein, high-carbohydrate diet. Muscle Heart
Mitochondria L2MBA BCKDHB BCKDHA DLD HMGCL BCKDHB BCKDHB BCAT1 BCAT1 BCAT1 BCKDHA BCKDHB DBT IVD ACADM ECHS1 MCCC1 MCCC2 DLD DBT BCKDHA BCKDHB BCKDHA BCKDHB DBT ACADS ACADSB ACAD8 ACADM ECHS1 HIBCH HIBADH ECHS1 ABAT ACADM ACADS ACADSB ECHS1 HSD17B10 ACAA2 ALDH6A1 PCCA PCCB MCEE MUT AOX1 ALDH2 AUH HMGCS2 ACAT1 OXCT1 BCKDHA BCKDHA Ac-CoA PropCoA Ac-CoA Suc-CoA HMG-CoA HMG-CoA 3MhThPP Lipoamd S3MdipE DHLipoa NAD NADH H+ LIN 2M1ThPP S2MDE Lipoamd 2M1ThPP S2MD ThiamPP Val AKG Ketoval Glu Leu AKG Ketoleu Glu Ile AKG 3M2Oxva Glu ThiamPP CO2 CoA IsovCoA 3McroCA HIV-CoA H2O ATP HCO3 3MCoA ADP DHLipoa NAD NADH H+ CoA IsoBCoA CO2 CO2 CoA 2IbuCoA McryCoA H2O 3HIsoCA H2O 3-HIBA CoA NAD 22M3O NADH H+ AKG Glu Tig-CoA H2O 2M3HC NAD 2Ma-CoA NADH CoA H2O NAD CoA NADH HCO3 ATP HCO3 S-MmCoA ADP Pi R-MmCoA H2O O2 Isosca H2O2 NAD H2O NADH H2O ActaCoA H2O CoA CoA Suc-CoA Succini ETF RETF ETF RETF ETF RETF PW000160 PW000005 PW000149 PW000005 PW000005