EC: 2.3.1.9

acetyl-CoA C-acetyltransferase (acetyl-CoA:acetyl-CoA C-acetyltransferase)

enzyme lambda metabolic reaction experiment
uniprot:Q9TUF6 [acetyl-CoA C-acetyltransferase(Enzyme) wildtype]

Acetoacetyl_CoA + Coenzyme_A -> 2 Acetyl_CoA		 (( (Vmax * S) ) / (Km + S)) buffer: 0.1 M Tris-HCl, 25 mM MgCl2
PH: 8.3
Temperature: 25
uniprot:Q5XI22 [acetyl-CoA C-acetyltransferase(Enzyme) wildtype]

Coenzyme_A + Acetoacetyl_CoA -> 2 Acetyl_CoA		 (( (Vmax * B) ) / (Km + B)) buffer: 50 mM Tris/HCl
PH: 8
Temperature: 37
uniprot:Q5XI22 [acetyl-CoA C-acetyltransferase(Enzyme) wildtype]

Acetoacetyl_CoA + Coenzyme_A -> 2 Acetyl_CoA		 (( (Vmax * A) ) / (Km + A)) buffer: 50 mM Tris/HCl
PH: 8
Temperature: 37
uniprot:Q5XI22 [acetyl-CoA C-acetyltransferase(Enzyme) wildtype]

2 Acetyl_CoA -> Coenzyme_A + Acetoacetyl_CoA		 (( (Vmax * A) ) / (Km + A)) buffer: 50 mM Tris/HCl, 0.1 mM NADH, 0.5 U/ml 3-hydroxyacyl-CoA dehydrogenase
PH: 8.8
Temperature: 37
uniprot:P24752 [acetyl-CoA C-acetyltransferase(Enzyme) wildtype His6-tagged]

H2O + Oleoyl_CoA -> Coenzyme_A + Oleate		 (( (Vmax * (S ^ n)) ) / (( (S_half ^ n) ) + (S ^ n))) buffer: 50 mM Tris, 1 mM EDTA, 10 nmol bovine serum albumin
PH: 7.8
Temperature: 37
uniprot:P24752 [acetyl-CoA C-acetyltransferase(Enzyme) mutant His6-tagged C528A, C546A]

H2O + Oleoyl_CoA -> Coenzyme_A + Oleate		 (( (Vmax * (S ^ n)) ) / (( (S_half ^ n) ) + (S ^ n))) buffer: 50 mM Tris, 1 mM EDTA, 10 nmol bovine serum albumin
PH: 7.8
Temperature: 37
uniprot:P24752 [acetyl-CoA C-acetyltransferase(Enzyme) mutant His6-tagged C528A]

H2O + Oleoyl_CoA -> Coenzyme_A + Oleate		 (( (Vmax * (S ^ n)) ) / (( (S_half ^ n) ) + (S ^ n))) buffer: 50 mM Tris, 1 mM EDTA, 10 nmol bovine serum albumin
PH: 7.8
Temperature: 37
uniprot:P24752 [acetyl-CoA C-acetyltransferase(Enzyme) mutant His6-tagged C92A, C333A, C345A, C365A, C387A, C467A, C516A, C528A, C546A]

Oleoyl_CoA + H2O -> Oleate + Coenzyme_A		 (( (Vmax * (S ^ n)) ) / (( (S_half ^ n) ) + (S ^ n))) buffer: 50 mM Tris, 1 mM EDTA, 10 nmol bovine serum albumin
PH: 7.8
Temperature: 37
uniprot:Q56WD9 [acetyl-CoA C-acetyltransferase(Enzyme) wildtype]

Coenzyme_A + Acetoacetyl_CoA -> 2 Acetyl_CoA		 (( (Vmax * S) ) / (Km + S)) buffer: *
PH: 7
Temperature: 36
uniprot:Q56WD9 [acetyl-CoA C-acetyltransferase(Enzyme) wildtype His-tagged, lacking N-terminal PTS2]

Acetoacetyl_CoA + Coenzyme_A -> 2 Acetyl_CoA		 (( (Vmax * S) ) / (Km + S)) buffer: *
PH: 7
Temperature: 36
uniprot:P17764 [acetyl-CoA C-acetyltransferase(Enzyme) wildtype isoenzyme Acat1]

Coenzyme_A + Acetoacetyl_CoA -> 2 Acetyl_CoA		 (( (Vmax * S1) ) / (Km1 + S1)) buffer: 100 mM Tris-HCl, 25 mM MgCl2, 50 mM KCl
PH: 8.1
Temperature: 30
uniprot:Q5XI22 [acetyl-CoA C-acetyltransferase(Enzyme) wildtype isoenzyme Acat2]

Acetoacetyl_CoA + Coenzyme_A -> 2 Acetyl_CoA		 (( (Vmax * S2) ) / (Km2 + S2)) buffer: 100 mM Tris-HCl, 25 mM MgCl2, 50 mM KCl
PH: 8.1
Temperature: 30
uniprot:Q5XI22 [acetyl-CoA C-acetyltransferase(Enzyme) wildtype isoenzyme Acat2]

Acetoacetyl_CoA + Coenzyme_A -> 2 Acetyl_CoA		 (( (Vmax * S1) ) / (Km1 + S1)) buffer: 100 mM Tris-HCl, 25 mM MgCl2, 50 mM KCl
PH: 8.1
Temperature: 30
uniprot:P17764 [K+]

Acetoacetyl_CoA + Coenzyme_A -> 2 Acetyl_CoA		 (( (Vmax * A) ) / (Ka + A)) buffer: 100 mM Tris-HCl, 25 mM MgCl2
PH: 8.1
Temperature: 30
uniprot:P17764 [acetyl-CoA C-acetyltransferase(Enzyme) wildtype isoenzyme Acat1]

Acetoacetyl_CoA + Coenzyme_A -> 2 Acetyl_CoA		 (( (Vmax * S2) ) / (Km2 + S2)) buffer: 100 mM Tris-HCl, 25 mM MgCl2, 50 mM KCl
PH: 8.1
Temperature: 30
- [N-Ethylmaleimide]

2 Acetyl_CoA -> Coenzyme_A + Acetoacetyl_CoA		 (( (Vmax * A) ) / (Km + A)) buffer: 50 mM Tris-HCl, 70 µM NADH, 2 U/ml 3-Hydroxyacyl-CoA dehydrogenase (EC 1.1.1.35)
PH: 8.1
Temperature: 30
- [acetyl-CoA C-acetyltransferase(Enzyme) wildtype]

2 Acetyl_CoA -> Coenzyme_A + Acetoacetyl_CoA		 (( (Vmax * A) ) / (Km + A)) buffer: 50 mM Tris-HCl, 70 µM NADH, 2 U/ml 3-Hydroxyacyl-CoA dehydrogenase (EC 1.1.1.35)
PH: 8.1
Temperature: 30
- [acetyl-CoA C-acetyltransferase(Enzyme) wildtype]

2 Acetyl_CoA -> Coenzyme_A + Acetoacetyl_CoA		 (( (Vmax * A) ) / (Km + A)) buffer: 50 mM Tris-HCl, 70 µM NADH, 2 U/ml 3-Hydroxyacyl-CoA dehydrogenase (EC 1.1.1.35)
PH: 8.1
Temperature: 30
- [acetyl-CoA C-acetyltransferase(Enzyme) wildtype]

2 Acetyl_CoA -> Acetoacetyl_CoA + Coenzyme_A		 (( (Vmax * A) ) / (Km + A)) buffer: 50 mM Tris-HCl, 70 µM NADH, 2 U/ml 3-Hydroxyacyl-CoA dehydrogenase (EC 1.1.1.35)
PH: 8.1
Temperature: 30
uniprot:P45359 [acetyl-CoA C-acetyltransferase(Enzyme) wildtype]

Coenzyme_A + Acetoacetyl_CoA -> 2 Acetyl_CoA		 (( (( (Vmax * A) ) * B) ) / (( (( (KmB * A) ) + (KmA * B)) ) + (A * B))) buffer: 100 mM Tris hydrochloride, 10 mM MgSO4, 1 mM dithiothreitol
PH: 8
Temperature: 30
uniprot:P45359 [acetyl-CoA C-acetyltransferase(Enzyme) wildtype]

2 Acetyl_CoA -> Coenzyme_A + Acetoacetyl_CoA		 (( (Vmax * A) ) / (Km + A)) buffer: 100 mM Tris hydrochloride, 1 mM dithiothreitol, 0.2 mM NADH, 2 U/ml beta-hydroxyacyl-CoA dehydrogenase
PH: 7.4
Temperature: 30

Pathways

pathway id name
BioCyc:HUMAN_PWY66-367 ketogenesis
BioCyc:HUMAN_PWY-5177 glutaryl-CoA degradation
BioCyc:HUMAN_ILEUDEG-PWY isoleucine degradation
BioCyc:HUMAN_TRYPTOPHAN-DEGRADATION-1 tryptophan degradation
BioCyc:HUMAN_PWY66-401 superpathway of tryptophan utilization
BioCyc:META_PWY-6863 pyruvate fermentation to hexanol (engineered)
BioCyc:META_PWY-6883 pyruvate fermentation to butanol II (engineered)
BioCyc:META_PWY-6886 1-butanol autotrophic biosynthesis (engineered)
BioCyc:META_FAO-PWY fatty acid β-oxidation I
BioCyc:META_PWY-922 mevalonate pathway I
BioCyc:META_ILEUDEG-PWY L-isoleucine degradation I
BioCyc:MTBCDC1551_ILEUDEG-PWY L-isoleucine degradation I
BioCyc:MOUSE_PWY66-368 ketolysis
BioCyc:MOUSE_PWY3FA-1022 ketone oxidation
BioCyc:MOUSE_PWY66-5 superpathway of cholesterol biosynthesis
BioCyc:MOUSE_PWY66-367 ketogenesis
BioCyc:MOUSE_PWY-5177 glutaryl-CoA degradation
BioCyc:GCF_000013425_FAO-PWY fatty acid β-oxidation I
BioCyc:HUMAN_PWY66-5 superpathway of cholesterol biosynthesis
BioCyc:HUMAN_PWY-5910 superpathway of geranylgeranyldiphosphate biosynthesis I (via mevalonate)
BioCyc:META_PWY-6604 superpathway of Clostridium acetobutylicum acidogenic and solventogenic fermentation
BioCyc:META_PWY-6588 pyruvate fermentation to acetone
BioCyc:META_PWY-6594 superpathway of Clostridium acetobutylicum solventogenic fermentation
BioCyc:META_CENTFERM-PWY pyruvate fermentation to butanoate
BioCyc:META_PWY-5177 glutaryl-CoA degradation
BioCyc:META_PWY-7003 glycerol degradation to butanol
BioCyc:META_PWY66-368 ketolysis
BioCyc:YEAST_ERGOSTEROL-SYN-PWY superpathway of ergosterol biosynthesis
BioCyc:YEAST_PWY-5910 superpathway of geranylgeranyldiphosphate biosynthesis I (via mevalonate)
BioCyc:YEAST_PWY-922 mevalonate pathway
BioCyc:MOUSE_PWY3DJ-0 isoleucine degradation
BioCyc:ECOL316407_ACETOACETATE-DEG-PWY acetoacetate degradation (to acetyl CoA)
BioCyc:HUMAN_PWY66-368 ketolysis
BioCyc:META_PWY-6876 isopropanol biosynthesis (engineered)
BioCyc:META_PWY1-3 polyhydroxybutanoate biosynthesis
BioCyc:META_PWY-7216 (R)- and (S)-3-hydroxybutanoate biosynthesis (engineered)
BioCyc:META_PWY-7218 photosynthetic 3-hydroxybutanoate biosynthesis (engineered)
BioCyc:META_PWY-5327 superpathway of L-lysine degradation
BioCyc:META_PWY-5910 superpathway of geranylgeranyldiphosphate biosynthesis I (via mevalonate)
BioCyc:META_PWY-6583 pyruvate fermentation to butanol I
BioCyc:ECO_ACETOACETATE-DEG-PWY acetoacetate degradation (to acetyl CoA)
BioCyc:MTBH37RV_ILEUDEG-PWY L-isoleucine degradation I
BioCyc:MTBCDC1551_PWY-5676 acetyl-CoA fermentation to butanoate II
BioCyc:MTBCDC1551_PWY-5177 glutaryl-CoA degradation
BioCyc:MOUSE_PWY-5109 2-methylbutyrate biosynthesis
BioCyc:PDIF272563_CENTFERM-PWY pyruvate fermentation to butanoate
BioCyc:HUMAN_PWY-922 mevalonate pathway
BioCyc:HUMAN_FAO-PWY fatty acid β-oxidation
BioCyc:HUMAN_PWY66-391 fatty acid β-oxidation (peroxisome)
BioCyc:META_ACETOACETATE-DEG-PWY acetoacetate degradation (to acetyl CoA)
BioCyc:META_PWY-6590 superpathway of Clostridium acetobutylicum acidogenic fermentation
BioCyc:META_PWY66-367 ketogenesis
BioCyc:META_PWY66-391 fatty acid β-oxidation VI (peroxisome)
BioCyc:META_ERGOSTEROL-SYN-PWY superpathway of ergosterol biosynthesis I
BioCyc:META_PWY66-5 superpathway of cholesterol biosynthesis
BioCyc:META_TRYPTOPHAN-DEGRADATION-1 L-tryptophan degradation III (eukaryotic)
BioCyc:MOUSE_FAO-PWY fatty acid β-oxidation I
BioCyc:MOUSE_TRYPTOPHAN-DEGRADATION-1 tryptophan degradation III (eukaryotic)
BioCyc:MOUSE_PWY3FA-941 ketogenesis
BioCyc:MOUSE_ACETOACETATE-DEG-PWY acetoacetate degradation (to acetyl CoA)
BioCyc:MOUSE_PWY-922 mevalonate pathway I
BioCyc:MOUSE_PWY-5136 fatty acid β-oxidation II (core pathway)
BioCyc:PDIF272563_PWY66-368 ketolysis
PathBank:SMP0002102 Acetate Metabolism
PlantCyc:PLANT_PWY-922 mevalonate pathway I
PlantCyc:PLANT_PWY-5910 superpathway of geranylgeranyldiphosphate biosynthesis I (via mevalonate)
PlantCyc:PLANT_LYSINE-DEG2-PWY lysine degradation II
Reactome:R-DRE-9854311 Maturation of TCA enzymes and regulation of TCA cycle
Reactome:R-MMU-9854311 Maturation of TCA enzymes and regulation of TCA cycle
Reactome:R-RNO-9854311 Maturation of TCA enzymes and regulation of TCA cycle
Reactome:R-BTA-9854311 Maturation of TCA enzymes and regulation of TCA cycle
Reactome:R-HSA-9854311 Maturation of TCA enzymes and regulation of TCA cycle
Reactome:R-HSA-9969896 Lanosterol biosynthesis
Reactome:R-MMU-9969896 Lanosterol biosynthesis
Reactome:R-RNO-9969896 Lanosterol biosynthesis
Reactome:R-BTA-1430728 Metabolism
Reactome:R-BTA-556833 Metabolism of lipids
Reactome:R-BTA-77108 Ketone body catabolism
Reactome:R-BTA-1428517 Aerobic respiration and respiratory electron transport
Reactome:R-BTA-71403 Citric acid cycle (TCA cycle)
Reactome:R-BTA-71291 Amino acid and derivative metabolism
Reactome:R-DRE-74182 Ketone body metabolism
Reactome:R-DRE-71291 Amino acid and derivative metabolism
Reactome:R-DDI-77111 Synthesis of Ketone Bodies
Reactome:R-HSA-2046106 alpha-linolenic acid (ALA) metabolism
Reactome:R-HSA-77108 Ketone body catabolism
Reactome:R-HSA-70895 Branched-chain amino acid catabolism
Reactome:R-HSA-9033500 TYSND1 cleaves peroxisomal proteins
Reactome:R-MMU-1430728 Metabolism
Reactome:R-MMU-556833 Metabolism of lipids
Reactome:R-MMU-2046106 alpha-linolenic acid (ALA) metabolism
Reactome:R-MMU-8957322 Metabolism of steroids
Reactome:R-MMU-77111 Synthesis of Ketone Bodies
Reactome:R-MMU-70895 Branched-chain amino acid catabolism
Reactome:R-RNO-8978868 Fatty acid metabolism
Reactome:R-RNO-2046104 alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
Reactome:R-RNO-77289 Mitochondrial Fatty Acid Beta-Oxidation
Reactome:R-RNO-74182 Ketone body metabolism
Reactome:R-RNO-70895 Branched-chain amino acid catabolism
Reactome:R-BTA-8978868 Fatty acid metabolism
Reactome:R-BTA-77289 Mitochondrial Fatty Acid Beta-Oxidation
Reactome:R-BTA-77111 Synthesis of Ketone Bodies
Reactome:R-BTA-70895 Branched-chain amino acid catabolism
Reactome:R-DRE-70895 Branched-chain amino acid catabolism
Reactome:R-DDI-74182 Ketone body metabolism
Reactome:R-DDI-71291 Amino acid and derivative metabolism
Reactome:R-HSA-556833 Metabolism of lipids
Reactome:R-HSA-8978868 Fatty acid metabolism
Reactome:R-HSA-2046104 alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
Reactome:R-HSA-77289 Mitochondrial Fatty Acid Beta-Oxidation
Reactome:R-HSA-191273 Cholesterol biosynthesis
Reactome:R-HSA-77111 Synthesis of Ketone Bodies
Reactome:R-HSA-9033241 Peroxisomal protein import
Reactome:R-MMU-8978868 Fatty acid metabolism
Reactome:R-MMU-2046104 alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
Reactome:R-MMU-77289 Mitochondrial Fatty Acid Beta-Oxidation
Reactome:R-MMU-74182 Ketone body metabolism
Reactome:R-RNO-390918 Peroxisomal lipid metabolism
Reactome:R-DRE-1430728 Metabolism
Reactome:R-DRE-556833 Metabolism of lipids
Reactome:R-DRE-77111 Synthesis of Ketone Bodies
Reactome:R-DRE-1428517 Aerobic respiration and respiratory electron transport
Reactome:R-DRE-71403 Citric acid cycle (TCA cycle)
Reactome:R-DDI-77108 Ketone body catabolism
Reactome:R-HSA-390247 Beta-oxidation of very long chain fatty acids
Reactome:R-HSA-1428517 Aerobic respiration and respiratory electron transport
Reactome:R-HSA-71403 Citric acid cycle (TCA cycle)
Reactome:R-HSA-71291 Amino acid and derivative metabolism
Reactome:R-HSA-9609507 Protein localization
Reactome:R-MMU-390247 Beta-oxidation of very long chain fatty acids
Reactome:R-MMU-191273 Cholesterol biosynthesis
Reactome:R-MMU-77108 Ketone body catabolism
Reactome:R-MMU-1428517 Aerobic respiration and respiratory electron transport
Reactome:R-MMU-71403 Citric acid cycle (TCA cycle)
Reactome:R-MMU-71291 Amino acid and derivative metabolism
Reactome:R-RNO-1430728 Metabolism
Reactome:R-RNO-556833 Metabolism of lipids
Reactome:R-RNO-2046106 alpha-linolenic acid (ALA) metabolism
Reactome:R-RNO-8957322 Metabolism of steroids
Reactome:R-RNO-77111 Synthesis of Ketone Bodies
Reactome:R-RNO-1428517 Aerobic respiration and respiratory electron transport
Reactome:R-RNO-71403 Citric acid cycle (TCA cycle)
Reactome:R-RNO-71291 Amino acid and derivative metabolism
Reactome:R-BTA-74182 Ketone body metabolism
Reactome:R-DRE-77108 Ketone body catabolism
Reactome:R-DDI-1430728 Metabolism
Reactome:R-DDI-556833 Metabolism of lipids
Reactome:R-DDI-70895 Branched-chain amino acid catabolism
Reactome:R-HSA-1430728 Metabolism
Reactome:R-HSA-390918 Peroxisomal lipid metabolism
Reactome:R-HSA-8957322 Metabolism of steroids
Reactome:R-HSA-74182 Ketone body metabolism
Reactome:R-MMU-390918 Peroxisomal lipid metabolism
Reactome:R-RNO-390247 Beta-oxidation of very long chain fatty acids
Reactome:R-RNO-191273 Cholesterol biosynthesis
Reactome:R-RNO-77108 Ketone body catabolism
WikiPathways:WP5175 Disorders in ketone body synthesis
WikiPathways:WP5195 Disorders in ketolysis
WikiPathways:WP5355 Metabolic Epileptic Disorders
WikiPathways:WP1941 Peroxisomal beta-oxidation of tetracosanoyl-CoA
WikiPathways:WP4742 Ketogenesis and ketolysis
WikiPathways:WP4686 Leucine, isoleucine and valine metabolism
WikiPathways:WP2318 Fatty acid oxidation
WikiPathways:WP4190 Mevalonate arm of cholesterol biosynthesis pathway
PathBank:SMP0120662 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I
PathBank:SMP0120726 Leigh Syndrome
PathBank:SMP0120734 Malonic Aciduria
PathBank:SMP0120746 Saccharopinuria/Hyperlysinemia II
PathBank:SMP0120786 Malonyl-CoA Decarboxylase Deficiency
PathBank:SMP0120794 Mevalonic Aciduria
PathBank:SMP0120806 3-Hydroxyisobutyric Aciduria
PathBank:SMP0120822 Carnitine Palmitoyl Transferase Deficiency I
PathBank:SMP0120826 Medium Chain Acyl-CoA Dehydrogenase Deficiency (MCAD)
PathBank:SMP0120842 Pyruvate Kinase Deficiency
PathBank:SMP0120854 Pyridoxine Dependency with Seizures
PathBank:SMP0120870 2-Aminoadipic 2-Oxoadipic Aciduria
PathBank:SMP0120440 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
PathBank:SMP0120444 3-Methylglutaconic Aciduria Type IV
PathBank:SMP0120516 Maple Syrup Urine Disease
PathBank:SMP0120589 Isovaleric Acidemia
PathBank:SMP0000032 Valine, Leucine, and Isoleucine Degradation
PathBank:SMP0000051 Fatty Acid Metabolism
PathBank:SMP0000016 Propanoate Metabolism
PathBank:SMP0000023 Steroid Biosynthesis
PathBank:SMP0000173 beta-Ketothiolase Deficiency
PathBank:SMP0000199 Maple Syrup Urine Disease
PathBank:SMP0000384 Methylmalonate Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0000387 CHILD Syndrome
PathBank:SMP0000235 Short-Chain Acyl-CoA Dehydrogenase Deficiency (SCAD Deficiency)
PathBank:SMP0000239 Saccharopinuria/Hyperlysinemia II
PathBank:SMP0000334 Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency)
PathBank:SMP0000482 Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids
PathBank:SMP0000141 3-Methylglutaconic Aciduria Type IV
PathBank:SMP0000112 Risedronate Action Pathway
PathBank:SMP0000508 Cholesteryl Ester Storage Disease
PathBank:SMP0000524 Isovaleric Acidemia
PathBank:SMP0000528 Hyperlysinemia II or Saccharopinuria
PathBank:SMP0000540 Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCAD)
PathBank:SMP0000544 Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD)
PathBank:SMP0000568 Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (SCHAD)
PathBank:SMP0002438 Cholesterol Biosynthesis and Metabolism CE(12:0)
PathBank:SMP0087185 Butyrate Metabolism
PathBank:SMP0087234 Valine, Leucine, and Isoleucine Degradation
PathBank:SMP0087294 Fatty Acid Elongation in Mitochondria
PathBank:SMP0087326 Steroid Biosynthesis
PathBank:SMP0087358 Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids
PathBank:SMP0120661 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
PathBank:SMP0120665 3-Methylglutaconic Aciduria Type IV
PathBank:SMP0120685 Chondrodysplasia Punctata II, X-Linked Dominant (CDPX2)
PathBank:SMP0120745 Methylmalonic Aciduria
PathBank:SMP0120753 Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency)
PathBank:SMP0120757 Propionic Acidemia
PathBank:SMP0120769 Hypercholesterolemia
PathBank:SMP0120793 Hyper-IgD Syndrome
PathBank:SMP0120805 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
PathBank:SMP0120825 Carnitine Palmitoyl Transferase Deficiency II
PathBank:SMP0120841 Primary Hyperoxaluria II, PH2
PathBank:SMP0120439 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency
PathBank:SMP0120443 3-Methylglutaconic Aciduria Type III
PathBank:SMP0120459 beta-Ketothiolase Deficiency
PathBank:SMP0120503 Isovaleric Aciduria
PathBank:SMP0120523 Methylmalonate Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0120588 Isobutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0120632 Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (SCHAD)
PathBank:SMP0121055 Mevalonate Pathway
PathBank:SMP0000071 Ketone Body Metabolism
PathBank:SMP0000073 Butyrate Metabolism
PathBank:SMP0000054 Fatty Acid Elongation in Mitochondria
PathBank:SMP0000138 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
PathBank:SMP0000140 3-Methylglutaconic Aciduria Type III
PathBank:SMP0000238 Isovaleric Aciduria
PathBank:SMP0000389 Smith-Lemli-Opitz Syndrome (SLOS)
PathBank:SMP0000319 Lysosomal Acid Lipase Deficiency (Wolman Disease)
PathBank:SMP0000185 Glutaric Aciduria Type I
PathBank:SMP0000186 Glutaric Aciduria Type I
PathBank:SMP0000196 Leigh Syndrome
PathBank:SMP0000082 Simvastatin Action Pathway
PathBank:SMP0000481 Mitochondrial Beta-Oxidation of Medium Chain Saturated Fatty Acids
PathBank:SMP0000201 Methylmalonic Aciduria Due to Cobalamin-Related Disorders
PathBank:SMP0000111 Cerivastatin Action Pathway
PathBank:SMP0000131 Atorvastatin Action Pathway
PathBank:SMP0000511 Wolman Disease
PathBank:SMP0000523 Isobutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0000527 Hyperlysinemia I, Familial
PathBank:SMP0000539 Long Chain Acyl-CoA Dehydrogenase Deficiency (LCAD)
PathBank:SMP0000559 Pyruvate Kinase Deficiency
PathBank:SMP0000571 Pyridoxine Dependency with Seizures
PathBank:SMP0002436 Cholesterol biosynthesis and metabolism CE(10:0)
PathBank:SMP0002441 Cholesterol biosynthesis and metabolism CE(18:0)
PathBank:SMP0087172 Fatty Acid Metabolism
PathBank:SMP0087265 Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids
PathBank:SMP0087325 Pyruvate Metabolism
PathBank:SMP0087341 Propanoate Metabolism
PathBank:SMP0063641 Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids
PathBank:SMP0063689 Valine, Leucine, and Isoleucine Degradation
PathBank:SMP0120660 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency
PathBank:SMP0120664 3-Methylglutaconic Aciduria Type III
PathBank:SMP0120684 CHILD Syndrome
PathBank:SMP0120692 Desmosterolosis
PathBank:SMP0120696 Ethylmalonic Encephalopathy
PathBank:SMP0120704 Glutaric Aciduria Type I
PathBank:SMP0120732 Lysosomal Acid Lipase Deficiency (Wolman Disease)
PathBank:SMP0120736 Maple Syrup Urine Disease
PathBank:SMP0120740 Short-Chain Acyl-CoA Dehydrogenase Deficiency (SCAD Deficiency)
PathBank:SMP0120748 Methylmalonic Aciduria Due to Cobalamin-Related Disorders
PathBank:SMP0120792 Cholesteryl Ester Storage Disease
PathBank:SMP0120808 Isovaleric Acidemia
PathBank:SMP0120812 Hyperlysinemia II or Saccharopinuria
PathBank:SMP0120824 Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCAD)
PathBank:SMP0120828 Trifunctional Protein Deficiency
PathBank:SMP0120852 Succinyl CoA: 3-Ketoacid CoA Transferase Deficiency
PathBank:SMP0070041 Cholesterol Biosynthesis and Metabolism
PathBank:SMP0120441 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I
PathBank:SMP0120525 Methylmalonic Aciduria
PathBank:SMP0120537 Propionic Acidemia
PathBank:SMP0120586 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
PathBank:SMP0000137 2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0000237 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I
PathBank:SMP0000200 Methylmalonic Aciduria
PathBank:SMP0000386 Desmosterolosis
PathBank:SMP0000212 Pyruvate Dehydrogenase Complex Deficiency
PathBank:SMP0000089 Pravastatin Action Pathway
PathBank:SMP0000095 Alendronate Action Pathway
PathBank:SMP0000099 Lovastatin Action Pathway
PathBank:SMP0000117 Pamidronate Action Pathway
PathBank:SMP0000509 Hyper-IgD Syndrome
PathBank:SMP0000521 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
PathBank:SMP0000541 Carnitine Palmitoyl Transferase Deficiency II
PathBank:SMP0000545 Trifunctional Protein Deficiency
PathBank:SMP0000569 Succinyl CoA: 3-Ketoacid CoA Transferase Deficiency
PathBank:SMP0000719 2-Aminoadipic 2-Oxoadipic Aciduria
PathBank:SMP0002434 Lysine Metabolism
PathBank:SMP0087203 Lysine Degradation
PathBank:SMP0087231 Pyruvate Metabolism
PathBank:SMP0087248 Propanoate Metabolism
PathBank:SMP0087280 Fatty Acid Metabolism
PathBank:SMP0087300 Lysine Degradation
PathBank:SMP0087360 Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids
PathBank:SMP0063640 Mitochondrial Beta-Oxidation of Medium Chain Saturated Fatty Acids
PathBank:SMP0120663 3-Methylglutaconic Aciduria Type I
PathBank:SMP0120679 beta-Ketothiolase Deficiency
PathBank:SMP0120723 Isovaleric Aciduria
PathBank:SMP0120735 Smith-Lemli-Opitz Syndrome (SLOS)
PathBank:SMP0120743 Methylmalonate Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0120751 Pyruvate Dehydrogenase Complex Deficiency
PathBank:SMP0120795 Wolman Disease
PathBank:SMP0120807 Isobutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0120811 Hyperlysinemia I, Familial
PathBank:SMP0120823 Long Chain Acyl-CoA Dehydrogenase Deficiency (LCAD)
PathBank:SMP0120827 Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD)
PathBank:SMP0120851 Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (SCHAD)
PathBank:SMP0120442 3-Methylglutaconic Aciduria Type I
PathBank:SMP0120587 3-Hydroxyisobutyric Aciduria
PathBank:SMP0121209 Mevalonate Pathway
PathBank:SMP0000037 Lysine Degradation
PathBank:SMP0000060 Pyruvate Metabolism
PathBank:SMP0000236 Propionic Acidemia
PathBank:SMP0000139 3-Methylglutaconic Aciduria Type I
PathBank:SMP0000388 Chondrodysplasia Punctata II, X-Linked Dominant (CDPX2)
PathBank:SMP0000181 Ethylmalonic Encephalopathy
PathBank:SMP0000079 Ibandronate Action Pathway
PathBank:SMP0000092 Rosuvastatin Action Pathway
PathBank:SMP0000480 Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids
PathBank:SMP0000198 Malonic Aciduria
PathBank:SMP0000209 Hypercholesterolemia
PathBank:SMP0000107 Zoledronate Action Pathway
PathBank:SMP0000119 Fluvastatin Action Pathway
PathBank:SMP0000502 Malonyl-CoA Decarboxylase Deficiency
PathBank:SMP0000510 Mevalonic Aciduria
PathBank:SMP0000522 3-Hydroxyisobutyric Aciduria
PathBank:SMP0000538 Carnitine Palmitoyl Transferase Deficiency I
PathBank:SMP0000542 Medium Chain Acyl-CoA Dehydrogenase Deficiency (MCAD)
PathBank:SMP0000558 Primary Hyperoxaluria II, PH2
PathBank:SMP0002355 Pyruvate Metabolism
PathBank:SMP0002435 Cholesterol biosynthesis and metabolism CE(14:0)
PathBank:SMP0002440 Cholesterol Biosynthesis and Metabolism CE(16:0)
PathBank:SMP0087202 Ketone Body Metabolism
PathBank:SMP0087287 Butyrate Metabolism
PathBank:SMP0087299 Ketone Body Metabolism
PathBank:SMP0087327 Valine, Leucine, and Isoleucine Degradation
PathBank:SMP0087359 Mitochondrial Beta-Oxidation of Medium Chain Saturated Fatty Acids
PathBank:SMP0012467 Butanoate Metabolism
PathBank:SMP0063639 Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids