| 1,1-Dimethylbiguanide Drug Metabolism Pathway |  | Metabolic | Metformin |
| 1,2-Benzodiazepine Drug Metabolism |  | Metabolic | 1,2-Benzodiazepine |
| 1-Methylhistidine Metabolism |  | Metabolic | Histidine / Ademetionine / ATP / beta-Alanine / 1-Methylhistidine |
| 11-beta-Hydroxylase Deficiency (CYP11B1) |  | Disease | NADH / Progesterone / Hydrocortisone / Mesoheme / Cholesterol / Corticosterone / 20-hydroxycholesterol |
| 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 |
| 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 |
| 2,2'-Dibenzothiazyl disulfide Drug Metabolism |  | Metabolic | 2,2'-Dibenzothiazyl disulfide |
| 2-Amino-3-Carboxymuconate Semialdehyde Degradation |  | Metabolic | NADH / Flavin adenine dinucleotide |
| 2-Aminoadipic 2-Oxoadipic Aciduria |  | Disease | Pyridoxal phosphate / Lysine / Glutamic acid / NADH / Oxogluric acid / Flavin adenine dinucleotide / L-Saccharopine |
| 2-Aminoadipic 2-Oxoadipic Aciduria |  | Disease | Pyridoxal phosphate / Lysine / Glutamic acid / NADH / Oxogluric acid / 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 / Oxogluric acid / Flavin adenine dinucleotide / gamma-Glutamylcysteine / Pyrophosphoric acid |
| 2-Ketoglutarate Dehydrogenase Complex Deficiency |  | Disease | Pyruvic acid / Biotin / Succinic acid / NADH / ATP / Magnesium cation / Oxogluric acid / Flavin adenine dinucleotide / Citric acid / Maleic acid |
| 2-Ketoglutarate Dehydrogenase Complex Deficiency |  | Disease | Pyruvic acid / Biotin / Succinic acid / NADH / ATP / Magnesium cation / Oxogluric acid / Flavin adenine dinucleotide / Citric acid / Maleic acid |
| 2-mercaptobenzothiazole Drug Metabolism |  | Metabolic | 2-mercaptobenzothiazole |
| 2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency |  | Disease | Pyridoxal phosphate / Biotin / Succinic acid / Glutamic acid / Pyridoxal / Leucine / NADH / Valine / Isoleucine / ATP / Acetoacetic acid / Oxogluric acid / Flavin adenine dinucleotide / alpha-Ketoisocaproic acid / alpha-Ketoisovalerate / Methylmalonic Acid |
| 2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency |  | Disease | Pyridoxal phosphate / Biotin / Succinic acid / Glutamic acid / Pyridoxal / Leucine / NADH / Valine / Isoleucine / ATP / Acetoacetic acid / Oxogluric 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 / Deoxycholic acid / Taurochenodeoxycholic acid |
| 3-beta-Hydroxysteroid Dehydrogenase Deficiency |  | Disease | NADH / Progesterone / Hydrocortisone / Mesoheme / Cholesterol / Corticosterone / 20-hydroxycholesterol |
| 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 / Oxogluric acid / Flavin adenine dinucleotide / alpha-Ketoisocaproic acid / alpha-Ketoisovalerate / Methylmalonic Acid |
| 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency |  | Disease | Pyridoxal phosphate / Biotin / Succinic acid / Glutamic acid / Pyridoxal / Leucine / NADH / Valine / Isoleucine / ATP / Acetoacetic acid / Oxogluric 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 / Oxogluric acid / Flavin adenine dinucleotide / alpha-Ketoisocaproic acid / alpha-Ketoisovalerate / Methylmalonic Acid |