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Metabolism and Physiological Effects of Citrulline
smpdb.ca/view/SMP0126861MetabolicIn addition, citrulline and L-aspartic acid can be converted into argininosuccinic acid through the action of the enzyme argininosuccinate synthase. … Citrulline has also been linked to several inborn metabolic disorders including argininosuccinic aciduria and fumarase deficiency.
Metabolism and Physiological Effects of Putrescine
smpdb.ca/view/SMP0123276MetabolicPutrescine is an aliphatic amine that is formed through gut microbial metabolism from the amino acid arginine which is acquired from foods that are high in protein. … After being transported into gut microbes, arginine undergoes 2 reactions with the enzymes Arginase and Ornithine Decarboxylase to form putrescine.
Metabolism and Physiological Effects of Xanthosine
smpdb.ca/view/SMP0124866MetabolicAdenosine then reacts with water to form inosine and ammonia using the enzyme adenosine deaminase. Inosine goes on to form hypoxanthine through the enzyme purine nucleoside phosphorylase. … Xanthosine is formed from purine catabolism. Purines can be made endogenously in the body or can be obtained exogenously from foods such as red meat. The purines are guanine and adenine.
Metabolism and Physiological Effects of Uridine
smpdb.ca/view/SMP0124871MetabolicL-glutamine is obtained form protein sources in the diet and is metabolized to uridine in the liver. … L-glutamine is first converted to carbamoyl phosphate then to N-Carbamoyl-L-aspartate and finally to dihydroorotate by the CAD protein (carbamoyl-phosphate synthetase 2, aspartate transcarbamylase, and
Enzymes: Neutral amino acid transporter B(0)Lysosomal 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. … Symptoms include anemia, diarrhea, vomiting, failure to thrive, enlarged liver, malabsorption, steatorrhea and abdominal pain.
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 1D-Arginine and D-Ornithine Metabolism
smpdb.ca/view/SMP0000036MetabolicD-Amino acids have been show to be present in high concentrations in humans and play a role in biological functions. … DAAO is highly specific towards D-amino acids and favours free neutral D-amino acids or those with hydrophobic, polar or aromatic groups. Acidic amino acids are not catalyze by DAOO.
Enzymes: D-amino-acid oxidaseMetabolism and Physiological Effects of Phenylacetylglutamine
smpdb.ca/view/SMP0123208MetabolicPhenylacetyl-CoA and L-glutamine react to form phenylacetylglutamine and coenzyme A. … acid.
Drugs: Phenylalanine · L-Glutamine · Adenosine phosphate · ATP · Magnesium cation · Phenylpyruvic acid +1 moreEnzymes: Y+L amino acid transporter 1, Excitatory amino acid transporter 3Metabolism and Physiological Effects of Phenol
smpdb.ca/view/SMP0123255MetabolicPhenol is shown to cause cardiovascular disease and enhance the production of reactive oxygen species and cause oxidative stress. … Phenol can get further metabolized in a liver hepatocyte to Phenyl sulphate and Phenyl glucuronide. However phenol itself is shown to be a major uremic toxin through high levels of retention.