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3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
smpdb.ca/view/SMP0125660Diseaseacid in urine; and ammonia in blood. … A deficiency in this enzyme results in accumulation of 3-hydroxymethylglutaric acid, 3-hydroxyisovaleric acid, 3-methylcrotonylglycine and 3-methylglutaconic acid (cis and trans form), and methylglutaric
Enzymes: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial, Propionyl-CoA carboxylase alpha chain, mitochondrial3-Methylcrotonyl-CoA Carboxylase Deficiency Type I
smpdb.ca/view/SMP0125662DiseaseSymptoms include recurring episodes of vomiting and diarrhea, lethargy, hypotonia, seizures, and coma. … As would naturally be expected, this causes the body to be unable to uptake and breakdown leucine properly.
Enzymes: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial, Propionyl-CoA carboxylase alpha chain, mitochondrialMethylmalonate Semialdehyde Dehydrogenase Deficiency
smpdb.ca/view/SMP0125669DiseaseSymptoms include failure to thrive, large liver, mental and motor retardation and vomiting. … methylmalonate semialdehydes to acetyl- and propionyl-CoA, respectively.
Enzymes: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial, Propionyl-CoA carboxylase alpha chain, mitochondrial3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
smpdb.ca/view/SMP0125673DiseaseThis 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. … Symptoms of the disorder include dysmorphic features, developmental delays and intellectual disabilities.
Enzymes: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial, Propionyl-CoA carboxylase alpha chain, mitochondrialGlutaric Aciduria Type I (Lysine Degradation)
smpdb.ca/view/SMP0125698DiseaseSymptoms include encephalopathy, grimacing, dystonia, metabolic acidosis, and hygroma. Treatment includes a low-protein diet, L-carnitine, riboflavin, and anticonvulsants. … A deficiency in this enzyme results in accumulation of 3-hydroxybutyric acid, 3-hydroxyglutaric acid, glutaconic acid, glutaric acid, and ketone bodies in urine.
Drugs: Pyridoxal phosphate · Lysine · Glutamic acid · NADH · Oxogluric acid · Flavin adenine dinucleotide +2 moreEnzymes: Medium-chain specific acyl-CoA dehydrogenase, mitochondrialBemiparin Action Pathway
smpdb.ca/view/SMP0126156Drug actionSome precautions is that Bemiparin may suppress the adrenal secretion of aldosterone and lead to elevated potassium. … It is excreted through hepatic and renal routes, elimination of the drug is impaired if there are issues with the renal or hepatic systems.
Drugs: CalciumEnzymes: Fibrinogen beta chain, Fibrinogen alpha chainSulodexide Action Pathway
smpdb.ca/view/SMP0126173Drug actionIt is eliminated through renal, fecal and bile routes, and sometimes adverse effects occur that relate to the gastrointestinal system. … Sulodexide mixture of glycosaminoglycans (GAGs), dermatan sulfate (DS) and fast-moving heparin.
Drugs: CalciumEnzymes: Fibrinogen beta chain, Fibrinogen alpha chainReviparin Action Pathway
smpdb.ca/view/SMP0126278Drug actionReviparin is a low molecular weight heparin derived from a pig's intestinal mucus and used to prevent thromboembolism.
Enzymes: Fibrinogen beta chain, Fibrinogen alpha chainArdeparin Action Pathway (new)
smpdb.ca/view/SMP0126657Drug actionDue to its anticoagulant nature herbs and supplements with similar activity should be avoided such as ginseng, ginkgo, ginger and garlic. … Some signs of overdose may include excessive bleeding and bruising.
Enzymes: Fibrinogen beta chain, Fibrinogen alpha chainAxitinib Action Pathway
smpdb.ca/view/SMP0126901Drug actionAxitinib is recognized for its high potency compared to earlier VEGFR inhibitors and is available in oral formulations under the brand name Inlyta®. … By blocking these receptors, axitinib hinders the process of angiogenesis (formation of new blood vessels), which is crucial for tumor growth and metastasis.
Enzymes: Fibrinogen beta chain, Fibrinogen alpha chain