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Metabolism and Physiological Effects of Isovalerylglycine
smpdb.ca/view/SMP0126748MetabolicIn certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. … Isovalerylglycine (IVG) is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids.
Metabolism and Physiological Effects of Tiglylglycine
smpdb.ca/view/SMP0126840MetabolicIn certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. … In the mitochondria, isoleucine is converted to 2-oxo-3-methylvaleric acid vis a transaminase. This is then converted into 2-methylbutyryl-CoA by the BCKADH complex.
Enzymes: Branched-chain-amino-acid aminotransferase, mitochondrial, Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrialMetabolism 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. … wax beans, and potato.
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. … While putrescine is important for interactions and processes involving, DNA, RNA and proteins, at high levels it is also a protein bound uremic toxin found in the body that can inhibit erythropoietin production
Metabolism and Physiological Effects of Xanthosine
smpdb.ca/view/SMP0124866MetabolicThe purines are guanine and adenine. These undergo metabolism in the liver to form xanthosine. Adenine forms adenosine through the enzyme purine nucleoside phosphorylase. … Xanthosine then enters the blood where it can have detrimental effects on other organs such as the kidney, causing renal failure and the heart causing cardiovascular disease.
Metabolism and Physiological Effects of Uridine
smpdb.ca/view/SMP0124871MetabolicMore specifically, uridine is a nucleoside consisting of uracil and D-ribose and a component of RNA. … 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. … Wolman disease is characterized by increased transaminases in serum, and increased cholesteryl esters and triglycerides in various tissues.
Drugs: Cholecalciferol · ATP · Magnesium cation · Iron · Dimethylallyl Diphosphate · Formic acid +8 moreEnzymes: Lysosomal acid lipase/cholesteryl ester hydrolase, Acetyl-CoA acetyltransferase, cytosolicMetabolism and Physiological Effects of N-alpha-Acetyl-L-arginine
smpdb.ca/view/SMP0125530MetabolicGlutamic acid is carboxylated into 1-Pyrroline-5-carboxylic acid by the enzyme Delta-1-pyrroline-5-carboxylate synthase. 1-Pyrroline-5-carboxylic acid with glutaric acid synthesize Oxoglutaric acid and … Arginnosuccinate is synthesized from citrulline and aspartic acid by the enzyme argininosuccinate synthase. That is catalyzed by argininosuccinate lyase to produce arginine and fumaric acid.
Drugs: Pyridoxal phosphate · Arginine · Aspartic acid · Ornithine · L-Glutamine · Adenosine phosphate +10 moreEnzymes: Neutral amino acid transporter A, Low affinity cationic amino acid transporter 2Congenital Bile Acid Synthesis Defect Type III
smpdb.ca/view/SMP0125690DiseaseThe 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 … CBASIII is characterized by accumulation of bile acids in the urine. Symptoms include severe cholestasis, cirrhosis, and liver failure.
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 1, Acetyl-CoA acetyltransferase, mitochondrial