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Fatty Acid Elongation in Mitochondria
smpdb.ca/view/SMP0000054MetabolicIn liver and kidney fatty acid elongation operates best in the presence of both NADH and NADPH, whereas in heart and skeletal muscle, only NADH is required. … There are 3 major processes that modify palmitic acid: elongation, desaturation and hydroxylation.
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. … The cyclooxygenase pathway begins in the cytosol with phospholipids being converted into arachidonic acid by the action of phospholipase A2.
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. … Wolman disease is characterized by increased transaminases in serum, and increased cholesteryl esters and triglycerides in various tissues.
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. … Wolman disease is characterized by increased transaminases in serum, and increased cholesteryl esters and triglycerides in various tissues.
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 1