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Monocarboxylate transporter 1 Pyruvate carboxylase, mitochondrial L-lactate dehydrogenase A-like 6A Pyruvate kinase isozymes R/L Phosphoenolpyruvate carboxykinase, cytosolic [GTP] Malate dehydrogenase, cytoplasmic NADP-dependent malic enzyme Probable D-lactate dehydrogenase, mitochondrial Hydroxyacylglutathione hydrolase, mitochondrial Lactoylglutathione lyase Glyoxylate reductase/hydroxypyruvate reductase Aldose reductase Aldose reductase NADP-dependent malic enzyme Dihydrolipoyl dehydrogenase E3 Dihydrolipoyl transacetylase E2 Acetyl-CoA carboxylase 1 Acetyl-CoA acetyltransferase, mitochondrial Acyl-coenzyme A thioesterase 12 Acylphosphatase- 1 Aldehyde dehydrogenase, mitochondrial Acetyl-coenzyme A synthetase, cytoplasmic Acetyl-coenzyme A synthetase, cytoplasmic Pyruvate dehydrogenase E1 Pyruvic acid Oxalacetic acid Phosphoenolpyruvic acid Pyruvaldehyde Acetyl-CoA Acetoacetyl-CoA L-Lactic acid L-Lactic acid ATP HCO3- ADP Pi NAD NADH H+ ATP ADP GTP GDP CO2 L-Malic acid NAD NADH H+ NAD CO2 NADH H+ D-Lactic acid H+ S-Lactoylglutathione H2O Glutathione Glutathione D-Lactaldehyde NAD NADH H+ Lactaldehyde NAD H+ NADH Propylene glycol NADP H+ NADPH NADP CO2 NADPH Dihydrolipoamide NAD Lipoamide NADH H+ S-Acetyldihydrolipoamide-E Coenzyme A ATP HCO3- Pi ADP Malonyl-CoA CoA CoA Acetic acid H2O Acetylphosphate H2O Pi Acetaldehyde NAD H2O NADH H+ ATP PPi Acetyl adenylate AMP PPi ATP CO2 Biotin Manganese Magnesium Potassium Manganese Manganese Zinc (II) ion Zinc (II) ion Manganese FAD Biotin Manganese Ferricytochrome c Ferrocytochrome c Nicotinate and Nicotinamide Metabolism Valine, Leucine, and Isoleucine Degradation Citric Acid Cycle Glycolysis Glycine and Serine Metabolism Propanoate Metabolism Ketone Body Metabolism Thiamine pyrophosphate Cytoplasm Mitochondria Mitochondrial matrix Mitochondria Mitochondrial Matrix Symptoms and signs include severe lactic acidosis in the newborns that usually leading to death, hypotonic, lethargic, seizures, mental retardation and spasticity.
Mitochondria Mitochondria SLC16A1 PC LDHAL6A PKLR PCK1 MDH1 ME1 LDHD HAGH GLO1 GRHPR AKR1B1 AKR1B1 ME1 DLD DLAT ACACA ACAT1 ACOT12 ACYP1 ALDH2 ACSS2 ACSS2 PDHA1 PDHB Pyruvic acid Oxalacetic acid Phosphoenolpyruvic acid Pyruvaldehyde Acetyl-CoA Acetoacetyl-CoA L-Lactic acid L-Lactic acid Adenosine triphosphate Hydrogen carbonate Adenosine diphosphate Phosphate NAD NADH Hydrogen Ion Adenosine triphosphate Adenosine diphosphate Guanosine triphosphate Guanosine diphosphate Carbon dioxide L-Malic acid NAD NADH Hydrogen Ion NAD Carbon dioxide NADH Hydrogen Ion D-Lactic acid Hydrogen Ion S- Lactoylglutathione Water Glutathione Glutathione D-Lactaldehyde NAD NADH Hydrogen Ion Lactaldehyde NAD Hydrogen NADH Propylene glycol NADP Hydrogen Ion NADPH NADP Carbon dioxide NADPH Dihydrolipoamide NAD Lipoamide NADH Hydrogen Ion S- Acetyldihydrolipoamide- E Coenzyme A Adenosine triphosphate Hydrogen carbonate Phosphoric acid Adenosine diphosphate Malonyl-CoA Coenzyme A Coenzyme A Acetic acid Water Acetylphosphate Water Phosphoric acid Acetaldehyde NAD Water NADH Hydrogen Ion Adenosine triphosphate Pyrophosphate Acetyl adenylate Adenosine monophosphate Pyrophosphate Adenosine triphosphate Carbon dioxide Ferricytochrome c Ferrocytochrome c Nicotinate and Nicotinamide Metabolism Valine, Leucine, and Isoleucine Degradation Citric Acid Cycle Glycolysis Glycine and Serine Metabolism Propanoate Metabolism Ketone Body Metabolism
SLC16A1 PC LDHAL6A PKLR PCK1 MDH1 ME1 LDHD HAGH GLO1 GRHPR AKR1B1 AKR1B1 ME1 DLD DLAT ACACA ACAT1 ACOT12 ACYP1 ALDH2 ACSS2 ACSS2 PDHA1 PDHB Pyr Oaa Pep Pyruval Ac-CoA ActaCoA Lactate Lactate ATP HCO3- ADP Pi NAD NADH H+ ATP ADP GTP GDP CO2 Malate NAD NADH H+ NAD CO2 NADH H+ D-Lac H+ S-LacGu H2O GSH GSH DL NAD NADH H+ LacAlhd NAD H+ NADH PGlycol NADP H+ NADPH NADP CO2 NADPH DHLipoa NAD Lipoamd NADH H+ SAE CoA ATP HCO3- Pi ADP MalyCoA CoA CoA Acoh H2O AcetylP H2O Pi Ethanal NAD H2O NADH H+ ATP PPi AAD AMP PPi ATP CO2 Biotin Mn2+ Mg2+ K+ Mn2+ Mn2+ Zinc Zinc Mn2+ FAD Biotin Mn2+ FC FC PW000151 PW000051 PW000005 PW000146 PW000157 PW000149 PW000028 ThiamPP Cytoplasm Mitochondria Mitochondrial matrix Mitochondria Mitochondrial Matrix Symptoms and signs include severe lactic acidosis in the newborns that usually leading to death, hypotonic, lethargic, seizures, mental retardation and spasticity.
Mitochondria Mitochondria SLC16A1 PC LDHAL6A PKLR PCK1 MDH1 ME1 LDHD HAGH GLO1 GRHPR AKR1B1 AKR1B1 ME1 DLD DLAT ACACA ACAT1 ACOT12 ACYP1 ALDH2 ACSS2 ACSS2 PDHA1 PDHB Pyr Oaa Pep Pyruval Ac-CoA ActaCoA Lactate Lactate ATP HCO3 ADP Pi NAD NADH H+ ATP ADP GTP GDP CO2 Malate NAD NADH H+ NAD CO2 NADH H+ D-Lac H+ S-LacGu H2O GSH GSH DL NAD NADH H+ LacAlhd NAD H2 NADH PGlycol NADP H+ NADPH NADP CO2 NADPH DHLipoa NAD Lipoamd NADH H+ SAE CoA ATP HCO3 PhosfrA ADP MalyCoA CoA CoA Acoh H2O AcetylP H2O PhosfrA Ethanal NAD H2O NADH H+ ATP Ppi AAD AMP Ppi ATP CO2 FC FC PW000151 PW000051 PW000005 PW000146 PW000157 PW000149 PW000028