| Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency) |  | Disease | Pyridoxal phosphate / Ademetionine / Arginine / Aspartic acid / Ornithine / Adenosine phosphate / Succinic acid / Glutamic acid / Glycine / Creatine / Citrulline / NADH / ATP / Proline / Sapropterin / Nitric Oxide / Mesoheme / Glycocyamine / 3,4-Dihydro-2h-Pyrrolium-5-Carboxylate / N(5)-[(hydroxyamino)(imino)methyl]-L-ornithine / Flavin adenine dinucleotide / Flavin mononucleotide / Urea / Pyrophosphoric acid / Maleic acid / 4-Carboxy-4-Aminobutanal |
| Argininemia |  | Disease | Pyridoxal phosphate / Pyruvic acid / Arginine / Aspartic acid / Ornithine / L-Glutamine / Adenosine phosphate / Glutamic acid / Citrulline / NADH / Alanine / ATP / Calcium / Urea / Pyrophosphoric acid / Maleic acid |
| Argininosuccinic Aciduria |  | Disease | Pyridoxal phosphate / Pyruvic acid / Arginine / Aspartic acid / Ornithine / L-Glutamine / Adenosine phosphate / Glutamic acid / Citrulline / NADH / Alanine / ATP / Calcium / Urea / Pyrophosphoric acid / Maleic acid |
| Aromatase Deficiency |  | Disease | NADH / Testosterone / Estrone / Estradiol / Calcium / Androstenedione / Formic acid / Mesoheme / Uridine-5'-Diphosphate / Estrone sulfate / Prasterone sulfate / 5beta-dihydrotestosterone |
| Aromatic L-Aminoacid Decarboxylase Deficiency |  | Disease | Pyridoxal phosphate / Ademetionine / Ascorbic acid / Tyrosine / Norepinephrine / Epinephrine / Dopamine / Levodopa / Iron / Pyrroloquinoline Quinone |
| Artemether Metabolism Pathway |  | Drug metabolism | NADH / Mesoheme / Uridine-5'-Diphosphate / Formaldehyde / Artemether |
| Aspartate Metabolism |  | Metabolic | Pyridoxal phosphate / Arginine / Aspartic acid / L-Glutamine / Adenosine phosphate / Glutamic acid / Citrulline / ATP / Asparagine / Magnesium cation / Zinc / D-Aspartic Acid / beta-Alanine / Flavin adenine dinucleotide / Acetic acid / Pyrophosphoric acid / N-Carbamoylaspartic acid / Maleic acid |
| Astemizole H1-Antihistamine Action |  | Drug action | Astemizole / Calcium |
| Atenolol Action Pathway |  | Drug action | ATP / Atenolol / Potassium cation / Calcium / Magnesium cation |
| Atorvastatin Action Pathway |  | Drug action | Cholecalciferol / ATP / Magnesium cation / Iron / Dimethylallyl Diphosphate / Formic acid / Geranyl Diphosphate / Mesoheme / Flavin adenine dinucleotide / Palmitic Acid / Pyrophosphoric acid / Cholesterol / ISOPENTENYL PYROPHOSPHATE / Farnesyl diphosphate |
| Azatadine H1-Antihistamine Action |  | Drug action | Azatadine / Calcium |
| Azathioprine Action Pathway |  | Drug action | Tetrahydrofolic acid / Ademetionine / Aspartic acid / L-Glutamine / Adenosine phosphate / Glutamic acid / Glycine / NADH / ATP / Adenine / Adenosine / Azathioprine / Mercaptopurine / Potassium cation / Calcium / Magnesium cation / Zinc / 5-O-phosphono-alpha-D-ribofuranosyl diphosphate / AICA ribonucleotide / Guanosine-5'-Monophosphate / Xanthine / Guanine / Cyclic adenosine monophosphate / Mesoheme / Guanosine / Ribose-1-Phosphate / Flavin adenine dinucleotide / Hypoxanthine / Pyrophosphoric acid / Maleic acid / Inosine / 2'-Deoxyguanosine-5'-Monophosphate |
| Azathioprine Metabolism Pathway |  | Drug metabolism | Azathioprine / Mercaptopurine |
| Azelastine H1-Antihistamine Action |  | Drug action | Azelastine / Calcium |
| Azithromycin Action Pathway |  | Drug action | Histidine / Adenosine phosphate / Methionine / Leucine / Threonine / Alanine / Valine / Isoleucine / ATP / Proline / Asparagine / Azithromycin / Guanosine-5'-Monophosphate / Cytidine-5'-Triphosphate / Uridine monophosphate / D-Serine / Pyrophosphoric acid |
| Bafetinib Inhibition of BCR-ABL |  | Drug action | Bafetinib |
| Bamipine H1-Antihistamine Action |  | Drug action | Calcium |
| Benazepril Action Pathway |  | Drug action | Benazepril / Zinc |
| Benazepril Metabolism Pathway |  | Drug metabolism | Benazepril / Zinc |
| Bendroflumethiazide Action Pathway |  | Drug action | Cysteine / Bendroflumethiazide / Potassium cation / Urea |
| Benzocaine Action Pathway |  | Drug action | ATP / Norepinephrine / Dopamine / Benzocaine / Potassium cation / Calcium |
| Bepotastine H1-Antihistamine Action |  | Drug action | Calcium / Bepotastine |
| Beta Oxidation of Very Long Chain Fatty Acids |  | Metabolic | ATP / Levocarnitine / Magnesium cation / Lauric acid |
| beta-Alanine Metabolism |  | Metabolic | Pyridoxal phosphate / Histidine / Aspartic acid / Glutamic acid / NADH / Calcium / Zinc / Pantothenic acid / Dihydrouracil / beta-Alanine / Flavin adenine dinucleotide / Flavin mononucleotide / Uracil |
| beta-Ketothiolase 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 |