S.No. Ailment Name Metabolism Cycle Enzyme Action
1 Maple syrup urine disease (MSUD) carbohydrate Inositol Phosphate Metabolism 1-phosphatidyl 1D-myo-inositol 3P?..1-phosphatidyl 1D-myo-inositol 1-3,5P2 Phosphoinositide kinase, FYVE finger containing (2.7.1.150) 2q34 View
2 Maple syrup urine disease (MSUD) carbohydrate Pentose and glucuronate interconversions ?-D-Glucuronoside?..D-Glucuronate Glucuronidase, beta (EC:3.2.1.31) 7q21.11 View
3 Maple syrup urine disease (MSUD) carbohydrate TCA Isocitrate??? oxalosuccinate Isocitrate Dehydrogenase (1.1.1.42,1.1.1.41) 2q33.3 View
4 Maple syrup urine disease (MSUD) lipid Steroid Hormone Biosynthesis cytochrome P450, family 11, subfamily B, polypeptide 2 (EC:1.14.15.4 1.14.15.5) View
5 Maple syrup urine disease (MSUD) lipid Steroid Hormone Biosynthesis CYP11B2 View
6 Maple syrup urine disease (MSUD) vitamin Pantothenate and CoA Biosynthesis Metabolism N-Pantothenoylcysteine... (R)-4'-Phosphopantothenoyl-L-cysteine Pantothenate kinase 1 (2.7.1.33) 10q23.31 View
7 Maple syrup urine disease (MSUD) vitamin 5 B6 METABOLISM Pyridoxine phosphate??...Pyridoxal 5-phosphate Pyridoxamine 5'-phosphate oxidase (1.4.3.5) 17q21.32 View
S.No Ailment Name Metabolism Cycle Metabolite Action
1 Maple syrup urine disease (MSUD) carbohydrate Butanoate Metabolism 4- Hydroxybutanoate View
2 Maple syrup urine disease (MSUD) carbohydrate Fructose and Mannose Degradation D - Sorbitol View
3 Maple syrup urine disease (MSUD) carbohydrate Galactose Metabolism Glucose View
4 Maple syrup urine disease (MSUD) carbohydrate Glycolysis Metabolism Glucose View
5 Maple syrup urine disease (MSUD) carbohydrate Pyruvate Metabolism Acetyl Co-A View
6 Maple syrup urine disease (MSUD) carbohydrate Pyruvate Metabolism Succinate View
7 Maple syrup urine disease (MSUD) lipid Primary and Secondry Bile Acid Biosynthesis Choles-5-ene-3?-26-diol (Oxysterol) View
8 Maple syrup urine disease (MSUD) lipid Glycero2 and Glycerophospho2 Metabolism Glycerate 3 phasphate View
9 Maple syrup urine disease (MSUD) lipid Glycero2 and Glycerophospho2 Metabolism View
10 Maple syrup urine disease (MSUD) lipid Glycero2 and Glycerophospho2 Metabolism Propionyl-CoA View
11 Maple syrup urine disease (MSUD) lipid Glycero2 and Glycerophospho2 Metabolism Propanal View
12 Maple syrup urine disease (MSUD) lipid Glycero2 and Glycerophospho2 Metabolism 1- Propanol View
13 Maple syrup urine disease (MSUD) lipid Synthesis and degradation of ketone bodies Acetyl Co-A View
14 Maple syrup urine disease (MSUD) lipid Sphingo2 Metabolism L-Serine View
15 Maple syrup urine disease (MSUD) lipid Steroid Hormone Biosynthesis 5?-Pregnane-3,20-dione View
16 Maple syrup urine disease (MSUD) nucleotide Adenine Metabolism Uric acid View
17 Maple syrup urine disease (MSUD) nucleotide Adenine Metabolism Carbamoyl phosphate View
18 Maple syrup urine disease (MSUD) nucleotide Guanine Metabolism (S)-2-[5-Amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido]succinate (SAICAR) View
19 Maple syrup urine disease (MSUD) nucleotide Thymine Metabolism Thymidine View
20 Maple syrup urine disease (MSUD) nucleotide Uracil Metabolism Ureidopropionic acid View
21 Maple syrup urine disease (MSUD) protein Alanine,Aspartate And Glutamate Metabolism L-Argino succinate View
22 Maple syrup urine disease (MSUD) protein Alanine,Aspartate And Glutamate Metabolism L-Glutamate View
23 Maple syrup urine disease (MSUD) protein Alanine,Aspartate And Glutamate Metabolism 4-Aminobutanoate View
24 Maple syrup urine disease (MSUD) protein Alanine,Aspartate And Glutamate Metabolism Succinate semialdehyde View
25 Maple syrup urine disease (MSUD) protein Arginine and Proline Metabolism Glutamine View
26 Maple syrup urine disease (MSUD) protein Arginine and Proline Metabolism L-Argino succinate View
27 Maple syrup urine disease (MSUD) protein Arginine and Proline Metabolism Arginine View
28 Maple syrup urine disease (MSUD) protein Arginine and Proline Metabolism Glyoxylate View
29 Maple syrup urine disease (MSUD) protein Cystein and Methionine Metabolism L-Serine View
30 Maple syrup urine disease (MSUD) protein Cystein and Methionine Metabolism L-Cystathionine View
31 Maple syrup urine disease (MSUD) protein Glycine, Serine And Threonine Metabolism L-Serine View
32 Maple syrup urine disease (MSUD) protein Glycine, Serine And Threonine Metabolism Cystathionine View
33 Maple syrup urine disease (MSUD) protein Glycine, Serine And Threonine Metabolism L-Tryptophane View
34 Maple syrup urine disease (MSUD) protein Glycine, Serine And Threonine Metabolism Glycine View
35 Maple syrup urine disease (MSUD) protein Histidine Metabolite Carnosine View
36 Maple syrup urine disease (MSUD) protein Phenylalanine,Tyrosine and Trytophan Biosynthesis Tyrosine View
37 Maple syrup urine disease (MSUD) protein Valine, leucine and isoleucine biosynthesis L- Valine View
38 Maple syrup urine disease (MSUD) protein Valine, leucine and isoleucine biosynthesis L- Leucine View
39 Maple syrup urine disease (MSUD) protein Valine, leucine and isoleucine biosynthesis L- Isoleucine View
40 Maple syrup urine disease (MSUD) protein Valine,Leucine and Isoleucine Degradation L- Isoleucine View
41 Maple syrup urine disease (MSUD) protein Valine,Leucine and Isoleucine Degradation L- Valine View
42 Maple syrup urine disease (MSUD) protein Valine,Leucine and Isoleucine Degradation L- Leucine View
43 Maple syrup urine disease (MSUD) protein Valine,Leucine and Isoleucine Degradation Acetyl Co-A View
44 Maple syrup urine disease (MSUD) vitamin Nicotinate and nicotinamide metabolism Quinolinate View
45 Maple syrup urine disease (MSUD) vitamin Nicotinate and nicotinamide metabolism Quinolinate View
46 Maple syrup urine disease (MSUD) vitamin Pantothenate and CoA biosynthesis L- Valine View
47 Maple syrup urine disease (MSUD) vitamin Pantothenate and CoA biosynthesis 3- methyl-2- oxobutanoate View
48 Maple syrup urine disease (MSUD) vitamin 5 A Metabolism Betacarotene View
49 Maple syrup urine disease (MSUD) vitamin Thiamine Metabolism Pyridoxal Phasphate View
50 Maple syrup urine disease (MSUD) vitamin Thiamine Metabolism Thiamine Phasphate View
51 Maple syrup urine disease (MSUD) vitamin Thiamine Metabolism Thiamine View
52 Maple syrup urine disease (MSUD) vitamin Thiamine Metabolism Glycine View
53 Maple syrup urine disease (MSUD) vitamin Thiamine Metabolism Tyrosine View
54 Maple syrup urine disease (MSUD) vitamin 5 D Metabolism Cholecalciferol (5 D3) View
55 Maple syrup urine disease (MSUD) vitamin 5 D Metabolism Cholecalciferol (5 D3) View
56 Maple syrup urine disease (MSUD) vitamin 5 D Metabolism Cholecalciferol (5 D3) View
57 Maple syrup urine disease (MSUD) vitamin 5 D Metabolism VDR View
58 Maple syrup urine disease (MSUD) vitamin 5 B6 Metabolism Pyridoxine View
59 Maple syrup urine disease (MSUD) vitamin 5 B6 Metabolism Pyridoxine View