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Teniposide Action Pathway
smpdb.ca/view/SMP0000443Drug actionTeniposide is a type of chemotherapy drug, derived from the epipodophyllotoxin form the American Mayapple plant. Teniposide is related to etoposide, another anti-cancer drug. It works in a similar way, inhibiting topoisomerase II. This c...
Refsum Disease
smpdb.ca/view/SMP0000451DiseaseAdult Refsum Disease (Classic Refsum Disease; Phytanic Acid Oxidase Deficiency; Heredopathia Atactica Polyneurtiformis; Hereditary Motor and Sensory Neuropathy IV; HSMN4; Adult Refsum Disease I; Adult Refsum Disease II), can be caused by...
Repaglinide Action Pathway
smpdb.ca/view/SMP0000454Drug actionRepaglinide is a non-sulfonylurea insulin secretagogue used in the treatment of type 2 diabetes. As the name of the drug class suggests, repaglinide acts on pancreatic beta-cells to stimulate insulin secretion. Under physiological condit...
Pyruvaldehyde Degradation
smpdb.ca/view/SMP0000459MetabolicThis Pyruvaldehyde degradation pathway (Methylglyoxal degradation;2-oxopropanal degradation), also known as the glyoxalase system, is probably the most common pathway for the degradation of pyruvaldehyde (methylglyoxal), a potentially to...
Drugs: Pyruvic acid · Glutathione · Zinc · D-Lactic acid · Flavin adenine dinucleotide · PyruvaldehydeUreidopropionase Deficiency
smpdb.ca/view/SMP0000492DiseaseHyper-beta-aminoisobutyric aciduria or BAIB urinary excretion is a common, autosomal recessive condition characterized by high levels of excretion of beta-aminoisobutyric acid in the urine. It is probably the most common mendelian metabo...
Galactosemia III
smpdb.ca/view/SMP0000496DiseaseGalactosemia III also called GALE deficiency or UDP-Galactose-4-Epimerase deficiency, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder caused by either a homozygous or compound heterozygous mutation in the UDP-...
beta-Mercaptolactate-Cysteine Disulfiduria
smpdb.ca/view/SMP0000499DiseaseMetcaptolactate-cysteine disulfiduria (MCDU) is an autosomal disorder that leads to the loss of function of the enzyme mercaptopyruvate sulfurtransferase. The condition is characterized by the urinary excretion of large amounts of a sulf...
Drugs: Pyridoxal phosphate · Pyruvic acid · Adenosine phosphate · Glutamic acid · Cysteine · NADH +6 moreMevalonic Aciduria
smpdb.ca/view/SMP0000510DiseaseMevalonic aciduria, also called MVA, is an extremely rare inherited inborn error of metabolism (IEM) and an autosomal recessive disorder caused by a defective mevalonate kinase gene. It is manifested by high levels of mevalonic acid in t...
Drugs: Cholecalciferol · ATP · Magnesium cation · Iron · Dimethylallyl Diphosphate · Formic acid +8 moreXanthinuria Type II
smpdb.ca/view/SMP0000513DiseaseXanthinuria Type II is a rare inborn error of metabolism (IEM) and autosomal recessive disorder and caused by a defective xanthine dehydrogenase. Xanthine dehydrogenase catalyzes the conversion of hypoxanthine into xanthine and conversio...
Drugs: Tetrahydrofolic acid · Aspartic acid · L-Glutamine · Adenosine phosphate · Glutamic acid · Glycine +24 moreGlucose-6-phosphate Dehydrogenase Deficiency
smpdb.ca/view/SMP0000518DiseaseGlucose-6-phosphate dehydrogenase deficiency, also called G6PDD, is a very common inherited inborn error of metabolism (IEM) that is characterized by a defect in the glucose-6-phosphate dehydrogenase gene. Glucose-6-phosphate dehydrogena...
Drugs: Adenosine phosphate · ATP · Magnesium cation · Iron · 5-O-phosphono-alpha-D-ribofuranosyl diphosphate · D-glyceraldehyde 3-phosphate +4 more