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3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
smpdb.ca/view/SMP0000521Diseaseacid. … This enzyme converts 2-methyl-3-oxopropanoate, CoA and water into propanoyl-CoA, using NAD+ as an oxidizing agent, and producing a hydrogen ion and hydrogencarbonate as byproducts.
Enzymes: Branched-chain-amino-acid aminotransferase, cytosolic, Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrialAcetylsalicylic Acid Action Pathway (New)
smpdb.ca/view/SMP0124628Drug actionAspirin (acetylsalicylic acid) is an NSAID used to treat pain, fever, inflammation, migraines, and reducing the risk of major adverse cardiovascular events. … It leads to the formation of prostaglandin E2 which is responsible for contributing to the inflammatory response by activating immune cells and for increasing pain sensation by acting on pain fibers.
Drugs: Glutathione · Dinoprostone · Acetylsalicylic acid · Calcium · Mesoheme · Prostaglandin G2 +1 moreNalidixic acid Predicted Metabolism Pathway
smpdb.ca/view/SMP0174549MetabolicMetabolites of Nalidixic acid are predicted with biotransformer.
Iocetamic acid Predicted Metabolism Pathway
smpdb.ca/view/SMP0174586MetabolicMetabolites of Iocetamic acid are predicted with biotransformer.
Lysosomal Acid Lipase Deficiency (Wolman Disease)
smpdb.ca/view/SMP0000319DiseaseLysosomal Acid Lipase Deficiency, also known as Wolman disease, is predictably enough the result of a specific defect in lysosomal acid lipase. … Symptoms include anemia, diarrhea, failure to thrive, enlarged liver, malabsorption, steatorrhea and abdominal pain.
Drugs: Cholecalciferol · ATP · Magnesium cation · Iron · Dimethylallyl Diphosphate · Formic acid +8 moreEnzymes: Lysosomal acid lipase/cholesteryl ester hydrolaseIndocyanine green acid form Drug Metabolism
smpdb.ca/view/SMP0130870MetabolicIndocyanine green acid form passes through the liver and is then excreted from the body mainly through the kidney. … Indocyanine green acid form is a drug that is not metabolized by the human body as determined by current research and biotransformer analysis.
Drugs: Indocyanine green acid formLysosomal Acid Lipase Deficiency (Wolman Disease)
smpdb.ca/view/SMP0125640DiseaseLysosomal Acid Lipase Deficiency, also known as Wolman disease, is predictably enough the result of a specific defect in lysosomal acid lipase. … Symptoms include anemia, diarrhea, vomiting, failure to thrive, enlarged liver, malabsorption, steatorrhea and abdominal pain.
Drugs: Cholecalciferol · ATP · Magnesium cation · Iron · Dimethylallyl Diphosphate · Formic acid +8 moreEnzymes: Lysosomal acid lipase/cholesteryl ester hydrolaseCongenital Bile Acid Synthesis Defect Type III
smpdb.ca/view/SMP0125690DiseaseCBASIII is characterized by accumulation of bile acids in the urine. Symptoms include severe cholestasis, cirrhosis, and liver failure. … The synthesis of primary bile acids from cholesterol occurs via two pathways: the classic neutral pathway involving cholesterol 7-alpha-hydroxylase (CYP7A1), and the acidic pathway involving a distinct
Glutaric Aciduria Type I (Fatty Acid Metabolism)
smpdb.ca/view/SMP0125737DiseaseA deficiency in this enzyme results in accumulation of 3-hydroxybutyric acid, 3-hydroxyglutaric acid, glutaconic acid, glutaric acid, and ketone bodies in urine. … Symptoms include encephalopathy, grimacing, dystonia, metabolic acidosis, and hygroma. Treatment includes a low-protein diet, L-carnitine, riboflavin, and anticonvulsants.
Drugs: Adenosine phosphate · NADH · ATP · Levocarnitine · Magnesium cation · Flavin adenine dinucleotide +4 moreEnzymes: Long-chain-fatty-acid--CoA ligase 1Congenital Bile Acid Synthesis Defect Type II
smpdb.ca/view/SMP0000314DiseaseChenodeoxycholic acid and cholic acid are decreased in plasma and urine. … Symptoms of this disease include cholestatic jaundice, atypical oxo and allo bile acids in urine and serum, liver failure, and steatosis.