| Hyper-IgD Syndrome |  | Disease | Cholecalciferol / ATP / Magnesium cation / Iron / Dimethylallyl Diphosphate / Formic acid / Geranyl Diphosphate / Mesoheme / Flavin adenine dinucleotide / Palmitic Acid / Pyrophosphoric acid / Cholesterol / ISOPENTENYL PYROPHOSPHATE / Farnesyl diphosphate |
| Hypercholesterolemia |  | Disease | Cholecalciferol / ATP / Magnesium cation / Iron / Dimethylallyl Diphosphate / Formic acid / Geranyl Diphosphate / Mesoheme / Flavin adenine dinucleotide / Palmitic Acid / Pyrophosphoric acid / Cholesterol / ISOPENTENYL PYROPHOSPHATE / Farnesyl diphosphate |
| Hypercholesterolemia |  | Disease | Cholecalciferol / ATP / Magnesium cation / Iron / Dimethylallyl Diphosphate / Formic acid / Geranyl Diphosphate / Mesoheme / Flavin adenine dinucleotide / Palmitic Acid / Pyrophosphoric acid / Cholesterol / ISOPENTENYL PYROPHOSPHATE / Farnesyl diphosphate |
| Hyperglycinemia, Non-Ketotic |  | 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 / Oxogluric acid / 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 |
| Hyperinsulinism-Hyperammonemia Syndrome |  | 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 |
| Hyperinsulinism-Hyperammonemia Syndrome |  | 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 |
| Hyperlysinemia I, Familial |  | Disease | Pyridoxal phosphate / Lysine / Glutamic acid / NADH / Oxogluric acid / Flavin adenine dinucleotide / L-Saccharopine |
| Hyperlysinemia I, Familial |  | Disease | Pyridoxal phosphate / Lysine / Glutamic acid / NADH / Oxogluric acid / Flavin adenine dinucleotide / L-Saccharopine |
| Hyperlysinemia II or Saccharopinuria |  | Disease | Pyridoxal phosphate / Lysine / Glutamic acid / NADH / Oxogluric acid / Flavin adenine dinucleotide / L-Saccharopine |
| Hypermethioninemia |  | Disease | Pyridoxal phosphate / Tetrahydrofolic acid / Ademetionine / Choline / Adenosine phosphate / Serine / Methionine / Glycine / Cysteine / NADH / ATP / Adenosine / Potassium cation / Magnesium cation / Zinc / Putrescine / N,N-dimethylglycine / 4-methylthio-2-oxobutanoic acid / Flavin adenine dinucleotide / Spermidine / Pyrophosphoric acid / L-Homoserine / 2-Oxobutanoic Acid |
| Hypermethioninemia |  | Disease | Pyridoxal phosphate / Tetrahydrofolic acid / Ademetionine / Choline / Adenosine phosphate / Serine / Methionine / Glycine / Cysteine / NADH / ATP / Adenosine / Potassium cation / Magnesium cation / Zinc / Putrescine / N,N-dimethylglycine / 4-methylthio-2-oxobutanoic acid / Flavin adenine dinucleotide / Spermidine / Pyrophosphoric acid / L-Homoserine / 2-Oxobutanoic Acid |
| Hyperornithinemia with Gyrate Atrophy (HOGA) |  | 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 / Oxogluric acid / N(5)-[(hydroxyamino)(imino)methyl]-L-ornithine / Flavin adenine dinucleotide / Flavin mononucleotide / Urea / Pyrophosphoric acid / Maleic acid / 4-Carboxy-4-Aminobutanal |
| Hyperornithinemia with Gyrate Atrophy (HOGA) |  | 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 / Oxogluric acid / N(5)-[(hydroxyamino)(imino)methyl]-L-ornithine / Flavin adenine dinucleotide / Flavin mononucleotide / Urea / Pyrophosphoric acid / Maleic acid / 4-Carboxy-4-Aminobutanal |
| Hyperornithinemia-Hyperammonemia-Homocitrullinuria (HHH-syndrome) |  | 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 / Oxogluric acid / N(5)-[(hydroxyamino)(imino)methyl]-L-ornithine / Flavin adenine dinucleotide / Flavin mononucleotide / Urea / Pyrophosphoric acid / Maleic acid / 4-Carboxy-4-Aminobutanal |
| Hyperornithinemia-Hyperammonemia-Homocitrullinuria (HHH-syndrome) |  | Disease | Pyridoxal phosphate / Ademetionine / Lysine / 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 / Oxogluric acid / N(5)-[(hydroxyamino)(imino)methyl]-L-ornithine / Flavin adenine dinucleotide / Flavin mononucleotide / Urea / Pyrophosphoric acid / Maleic acid / 4-Carboxy-4-Aminobutanal |
| Hyperphenylalaninemia Due to 6-Pyruvoyltetrahydropterin Synthase Deficiency (ptps) |  | Disease | Phenylalanine / Tyrosine / NADH / Sapropterin / Iron / Zinc / Formic acid / D-threo-neopterin / Triphosphoric acid |
| Hyperphenylalaninemia Due to 6-Pyruvoyltetrahydropterin Synthase Deficiency (ptps) |  | Disease | NADH / Sapropterin / Zinc / Formic acid / D-threo-neopterin / Triphosphoric acid |
| Hyperphenylalaninemia Due to DHPR-Deficiency |  | Disease | NADH / Sapropterin / Zinc / Formic acid / D-threo-neopterin / Triphosphoric acid |
| Hyperphenylalaninemia Due to DHPR-Deficiency |  | Disease | Phenylalanine / Tyrosine / NADH / Sapropterin / Iron / Zinc / Formic acid / D-threo-neopterin / Triphosphoric acid |
| Hyperphenylalaninemia Due to Guanosine Triphosphate Cyclohydrolase Deficiency |  | Disease | Phenylalanine / Tyrosine / NADH / Sapropterin / Iron / Zinc / Formic acid / D-threo-neopterin / Triphosphoric acid |
| Hyperphenylalaninemia Due to Guanosine Triphosphate Cyclohydrolase Deficiency |  | Disease | NADH / Sapropterin / Zinc / Formic acid / D-threo-neopterin / Triphosphoric acid |
| Hyperprolinemia Type I |  | 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 / Oxogluric acid / N(5)-[(hydroxyamino)(imino)methyl]-L-ornithine / Flavin adenine dinucleotide / Flavin mononucleotide / Urea / Pyrophosphoric acid / Maleic acid / 4-Carboxy-4-Aminobutanal |
| Hyperprolinemia Type I |  | 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 / Oxogluric acid / N(5)-[(hydroxyamino)(imino)methyl]-L-ornithine / Flavin adenine dinucleotide / Flavin mononucleotide / Urea / Pyrophosphoric acid / Maleic acid / 4-Carboxy-4-Aminobutanal |
| Hyperprolinemia Type II |  | 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 / Oxogluric acid / N(5)-[(hydroxyamino)(imino)methyl]-L-ornithine / Flavin adenine dinucleotide / Flavin mononucleotide / Urea / Pyrophosphoric acid / Maleic acid / 4-Carboxy-4-Aminobutanal |
| Hyperprolinemia Type II |  | 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 / Oxogluric acid / N(5)-[(hydroxyamino)(imino)methyl]-L-ornithine / Flavin adenine dinucleotide / Flavin mononucleotide / Urea / Pyrophosphoric acid / Maleic acid / 4-Carboxy-4-Aminobutanal |