| 11-beta-Hydroxylase Deficiency (CYP11B1) |  | Disease | NADH /Progesterone /Hydrocortisone /Mesoheme /Cholesterol /Corticosterone /20-hydroxycholesterol |
| 17-alpha-Hydroxylase Deficiency (CYP17) |  | Disease | NADH /Progesterone /Hydrocortisone /Mesoheme /Cholesterol /Corticosterone /20-hydroxycholesterol |
| 17-beta Hydroxysteroid Dehydrogenase III Deficiency |  | Disease | NADH /Testosterone /Estrone /Estradiol /Calcium /Androstenedione /Formic acid /Mesoheme /Uridine-5'-Diphosphate /Estrone sulfate /Prasterone sulfate /5beta-dihydrotestosterone |
| 2-Aminoadipic 2-Oxoadipic Aciduria |  | Disease | Pyridoxal phosphate /Lysine /Glutamic acid /NADH /Flavin adenine dinucleotide /L-Saccharopine |
| 2-Hydroxyglutric Aciduria (D and L Form) |  | Disease | Pyridoxal phosphate /Pyruvic acid /Biotin /Aspartic acid /L-Glutamine /Adenosine phosphate /Succinic acid /Glutamic acid /Glutathione /Glycine /Cysteine /NADH /Alanine /ATP /Magnesium cation /Zinc /5-O-phosphono-alpha-D-ribofuranosyl diphosphate /Guanosine-5'-Monophosphate /gamma-Aminobutyric acid /Flavin adenine dinucleotide /gamma-Glutamylcysteine /Pyrophosphoric acid |
| 2-Ketoglutarate Dehydrogenase Complex Deficiency |  | Disease | Pyruvic acid /Biotin /Succinic acid /NADH /ATP /Magnesium cation /Flavin adenine dinucleotide /Citric acid /Maleic acid |
| 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency |  | Disease | Pyridoxal phosphate /Biotin /Succinic acid /Glutamic acid /Pyridoxal /Leucine /NADH /Valine /Isoleucine /ATP /Acetoacetic acid /Flavin adenine dinucleotide /alpha-Ketoisocaproic acid /alpha-Ketoisovalerate /Methylmalonic Acid |
| 21-Hydroxylase Deficiency (CYP21) |  | Disease | NADH /Progesterone /Hydrocortisone /Mesoheme /Cholesterol /Corticosterone /20-hydroxycholesterol |
| 27-Hydroxylase Deficiency |  | Disease | Glycine /NADH /ATP /Iron /Taurine /Mesoheme /Cholic Acid /Flavin adenine dinucleotide /Palmitic Acid /Pyrophosphoric acid /Taurocholic acid /Cholesterol /Chenodeoxycholic acid /Taurochenodeoxycholic acid |
| 3-beta-Hydroxysteroid Dehydrogenase Deficiency |  | Disease | NADH /Progesterone /Hydrocortisone /Mesoheme /Cholesterol /Corticosterone /20-hydroxycholesterol |
| 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency |  | Disease | Pyridoxal phosphate /Biotin /Succinic acid /Glutamic acid /Pyridoxal /Leucine /NADH /Valine /Isoleucine /ATP /Acetoacetic acid /Flavin adenine dinucleotide /alpha-Ketoisocaproic acid /alpha-Ketoisovalerate /Methylmalonic Acid |
| 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency |  | Disease | Pyridoxal phosphate /Biotin /Succinic acid /Glutamic acid /Pyridoxal /Leucine /NADH /Valine /Isoleucine /ATP /Acetoacetic acid /Flavin adenine dinucleotide /alpha-Ketoisocaproic acid /alpha-Ketoisovalerate /Methylmalonic Acid |
| 3-Hydroxyisobutyric Aciduria |  | Disease | Pyridoxal phosphate /Biotin /Succinic acid /Glutamic acid /Pyridoxal /Leucine /NADH /Valine /Isoleucine /ATP /Acetoacetic acid /Flavin adenine dinucleotide /alpha-Ketoisocaproic acid /alpha-Ketoisovalerate /Methylmalonic Acid |
| 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I |  | Disease | Pyridoxal phosphate /Biotin /Succinic acid /Glutamic acid /Pyridoxal /Leucine /NADH /Valine /Isoleucine /ATP /Acetoacetic acid /Flavin adenine dinucleotide /alpha-Ketoisocaproic acid /alpha-Ketoisovalerate /Methylmalonic Acid |
| 3-Methylglutaconic Aciduria Type I |  | Disease | Pyridoxal phosphate /Biotin /Succinic acid /Glutamic acid /Pyridoxal /Leucine /NADH /Valine /Isoleucine /ATP /Acetoacetic acid /Flavin adenine dinucleotide /alpha-Ketoisocaproic acid /alpha-Ketoisovalerate /Methylmalonic Acid |
| 3-Methylglutaconic Aciduria Type III |  | Disease | Pyridoxal phosphate /Biotin /Succinic acid /Glutamic acid /Pyridoxal /Leucine /NADH /Valine /Isoleucine /ATP /Acetoacetic acid /Flavin adenine dinucleotide /alpha-Ketoisocaproic acid /alpha-Ketoisovalerate /Methylmalonic Acid |
| 3-Methylglutaconic Aciduria Type IV |  | Disease | Pyridoxal phosphate /Biotin /Succinic acid /Glutamic acid /Pyridoxal /Leucine /NADH /Valine /Isoleucine /ATP /Acetoacetic acid /Flavin adenine dinucleotide /alpha-Ketoisocaproic acid /alpha-Ketoisovalerate /Methylmalonic Acid |
| 3-Methylthiofentanyl Action Pathway |  | Drug action | ATP /Norepinephrine /Dopamine /Potassium cation /Calcium /3-Methylthiofentanyl |
| 3-Phosphoglycerate Dehydrogenase Deficiency |  | Disease | Pyridoxal phosphate /Tetrahydrofolic acid /Ademetionine /Pyruvic acid /Arginine /Adenosine phosphate /Serine /Methionine /Glutamic acid /Glycine /Creatine /Cysteine /NADH /Alanine /ATP /Aminolevulinic acid /Magnesium cation /Zinc /N,N-dimethylglycine /Glycocyamine /3-Hydroxypyruvic Acid /Orotidylic acid /Flavin adenine dinucleotide /Pyruvaldehyde /Formaldehyde /2-Amino-3-Ketobutyric Acid /D-Serine /Pyrophosphoric acid /Glyoxylic acid /Dexfosfoserine /2-Oxobutanoic Acid |
| 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency |  | Disease | Pyridoxal phosphate /Pyruvic acid /Biotin /Aspartic acid /L-Glutamine /Adenosine phosphate /Succinic acid /Glutamic acid /Glutathione /Glycine /Cysteine /NADH /Alanine /ATP /Magnesium cation /Zinc /5-O-phosphono-alpha-D-ribofuranosyl diphosphate /Guanosine-5'-Monophosphate /gamma-Aminobutyric acid /Flavin adenine dinucleotide /gamma-Glutamylcysteine /Pyrophosphoric acid |
| 5-Oxoprolinase Deficiency |  | Disease | Glutamic acid /Glutathione /Glycine /Cysteine /Alanine /ATP /Zinc /Pidolic acid /Flavin adenine dinucleotide /gamma-Glutamylcysteine /S-Methyl glutathione |
| 5-Oxoprolinuria |  | Disease | Glutamic acid /Glutathione /Glycine /Cysteine /Alanine /ATP /Zinc /Pidolic acid /Flavin adenine dinucleotide /gamma-Glutamylcysteine /S-Methyl glutathione |
| Abacavir Action Pathway |  | Drug action | Adenosine phosphate /ATP /Abacavir /Pyrophosphoric acid |
| Abciximab Action Pathway |  | Drug action | Abciximab |
| Acebutolol Action Pathway |  | Drug action | ATP /Acebutolol /Potassium cation /Calcium /Magnesium cation |