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beta-Alanine Metabolism
smpdb.ca/view/SMP0000007MetabolicBeta-alanine, 3-aminopropanoic acid, is a non-essential amino acid. Beta-Alanine is formed by the proteolytic degradation of beta-alanine containing dipeptides: carnosine, anserine, balenine, and pantothenic acid (vitamin B5). These dipe...
Glutaric Aciduria Type I
smpdb.ca/view/SMP0000186DiseaseTreatment includes a low-protein diet, L-carnitine, riboflavin, and anticonvulsants.
Drugs: Pyridoxal phosphate · Lysine · Glutamic acid · NADH · Oxogluric acid · Flavin adenine dinucleotide +1 moreProlidase Deficiency (PD)
smpdb.ca/view/SMP0000207DiseaseProlidase deficiency is an autosomal recessive disorder. The enzyme prolidase cleaves iminodipeptides with N-terminal proline or hydroxyproline. Collagen has high levels of iminoacids therefore, these dipeptidases are important for colla...
Drugs: Pyridoxal phosphate · Ademetionine · Arginine · Aspartic acid · Ornithine · Adenosine phosphate +21 moreUMP Synthase Deficiency (Orotic Aciduria)
smpdb.ca/view/SMP0000219DiseaseOrotic aciduria, also known as UMP synthase deficiency, is an autosomal recessive disorder of pyrimidine metabolism caused by a defective uridine monophosphate synthetase (UMPS). UMPS is a multifunctional protein which carries out the fu...
Hyperprolinemia Type II
smpdb.ca/view/SMP0000360DiseaseHyperprolinemia Type II (HPII), also known as 1-pyrroline-5-carboxylate dehydrogenase deficiency, is an extremely rare inborn error of metabolism (IEM) and autosomal recessive disorder of the arginine and proline metabolism pathway. It i...
Drugs: Pyridoxal phosphate · Ademetionine · Arginine · Aspartic acid · Ornithine · Adenosine phosphate +21 moreUreidopropionase 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...
Doxorubicin Metabolism Pathway
smpdb.ca/view/SMP0000650Drug metabolismDoxorubicin is an anthracycline antibiotic used as a cancer chemotherapy drug. The major metabolic route of doxorubicin metabolism is two-electron reduction to doxorubicinol. A second route is one-electron reduction resulting in a doxoru...
Mevalonate Pathway
smpdb.ca/view/SMP0121055MetabolicThe Mevalonate Pathway is a necessary pathway that occurs in archaea, eukaryotes and select bacteria. It has mainly been studied with regard to cholesterol biosynthesis and how it relates to cardiovascular disease in humans, but has rece...
Drugs: ATP · Magnesium cation · Dimethylallyl Diphosphate · Geranyl Diphosphate · Flavin adenine dinucleotide · Flavin mononucleotide +3 moreBloch Pathway (Cholesterol Biosynthesis)
smpdb.ca/view/SMP0121057MetabolicThe Bloch pathway, named after Konrad Bloch, is the pathway following the mevalonate pathway occurring within the cell to complete cholesterol biosynthesis. Cholesterol is a necessary metabolite that helps create many essential hormones ...
Drugs: Iron · Formic acid · Mesoheme · Flavin adenine dinucleotide · Flavin mononucleotide · CholesterolFluorouracil Metabolism
smpdb.ca/view/SMP0121111MetabolicFluorouracil, sold as Adrucil, Carac, Efudex, Efudix and others, is a medication used to treat various forms of cancer. It consists of a fluorine atom on the 5th carbon of a uracil molecule, and is treated similarly to the uracil during ...