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Thiamine Metabolism
smpdb.ca/view/SMP0000076MetabolicThiamine, (Vitamin B1), is a compound found in many different foods such as beans, seafood, meats and yogurt. It is usually maintained by the consumption of whole grains. It is an essential part of energy metabolism. This means that thia...
Alendronate Action Pathway
smpdb.ca/view/SMP0000095Drug actionAlendronate (also known as alendronic acid) is a type of bisphosphonate medication with nitrogen that can inhibit FPP synthase, which can block the pathway that produce geranyl-PP and farnesyl pyrophosphate. Geranyl-PP and farnesyl pyrop...
Drugs: Cholecalciferol · ATP · Alendronic acid · Magnesium cation · Iron · Dimethylallyl Diphosphate +9 moreZoledronate Action Pathway
smpdb.ca/view/SMP0000107Drug actionZoledronate (also named zoledronic acid, Zometa or Reclast) is a type of medication that used to treat numbers of bone diseases because of its affinity for hydroxyapatite. Zoledronate targets farnesyl pyrophosphate (FPP) synthase by inhi...
Drugs: Cholecalciferol · ATP · Zoledronic acid · Magnesium cation · Iron · Dimethylallyl Diphosphate +9 moreCerivastatin Action Pathway
smpdb.ca/view/SMP0000111Drug actionCerivastatin inhibits cholesterol synthesis via the mevalonate pathway by inhibiting 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase. HMG-CoA reductase is the enzyme responsible for the conversion of HMG-CoA to mevalonic acid, ...
Drugs: Cholecalciferol · ATP · Cerivastatin · Magnesium cation · Iron · Dimethylallyl Diphosphate +9 morebeta-Ureidopropionase Deficiency
smpdb.ca/view/SMP0000172DiseaseBeta-ureidopropionase deficiency (Beta Alanine-Synthase Deficiency, UPB1, BUP1) is an autosomal recessive disease caused by mutations in the UPB1 gene which codes for beta-ureidopropionase. A deficiency in this enzyme results in accumula...
MNGIE (Mitochondrial Neurogastrointestinal Encephalopathy)
smpdb.ca/view/SMP0000202DiseaseMyoneurogastrointestinal encephalopathy, or mitochondrial neurogastrointestinal encephalopathy syndrome (MNGIE), is a multisystem disorder caused by mutations in the gene encoding thymidine phosphorylase, which normally uses thymidine an...
Tyrosinemia Type I
smpdb.ca/view/SMP0000218DiseaseTyrosinemia type I, also known as fumarylacetoacetase or FAH deficiency, is the most severe type of tyrosinemia, a buildup of tyrosine in the body. It is caused by an autosomal recessive mutation in the the FAH gene that encodes for fuma...
Drugs: Pyridoxal phosphate · Ademetionine · Ascorbic acid · Aspartic acid · Tyrosine · Glutamic acid +20 moreChondrodysplasia Punctata II, X-Linked Dominant (CDPX2)
smpdb.ca/view/SMP0000388DiseaseChondrodysplasia Punctata 2, X Linked Dominant (CDPX2; CPDXD; CPXD; Conradi-Hunermann Syndrome; Happle Syndrome; Conradi-Hunermann-Happle Syndrome is caused by a mutation in the gene encoding delta(8)-delta(7) sterol isomerase emopamil-b...
Drugs: Cholecalciferol · ATP · Magnesium cation · Iron · Dimethylallyl Diphosphate · Formic acid +8 moreLactose Synthesis
smpdb.ca/view/SMP0000444MetabolicLactose synthesis occurs only in the mammary glands, producing lactose (4-O-B-D-galactosylpyranosyl-a-D-glucopyranoside), the major sugar in milk. Lactose is created by joining two monosaccarides with a B1,4 glycosidic bond. Glucose is f...
Galactosemia II (GALK)
smpdb.ca/view/SMP0000495DiseaseGalactokinase deficiency also called Galactosemia type II, is a rare inborn error of metabolism (IEM) and an autosomal recessive disorder of galactokinase caused by a mutation in the GALK1 gene on chromosome 17q24. Galactokinase uses 1 A...