| 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 |
