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Threonine and 2-Oxobutanoate Degradation
smpdb.ca/view/SMP0000452Metabolic2-oxobutanoate, also known as 2-Ketobutyric acid, is a 2-keto acid that is commonly produced in the metabolism of amino acids such as methionine and threonine. … Like other 2-keto acids, degradation of 2-oxobutanoate occurs in the mitochondrial matrix and begins with oxidative decarboxylation to its acyl coenzyme A derivative, propionyl-CoA.
Enzymes: Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrialMetabolism and Physiological Effects of Indoxyl glucoside (indican)
smpdb.ca/view/SMP0124742MetabolicIndican is then excreted into the urine and from there into the diaper where, upon exposure to air, it is converted to indigo blue dye due to oxidation by atmospheric oxygen. … It is also shown to cause vascular calcification and disrupt the electron transport chain and oxidative phosphorylation causing muscle atrophy.
Apoptotic DNA Fragmentation and Tissue Homeostasis
smpdb.ca/view/SMP0063772SignalingEndoG resides in mitochondria in normal cells and travels to the nucleus, where it fragments chromosomal DNA upon activation of apoptosis. … ICAD and CAD exist in the nucleus in normal cells and is a major endonuclease in apoptosis. Its activation is normally caspase dependent.
Drugs: ZincMetabolism and Physiological Effects of Creatinine
smpdb.ca/view/SMP0123283MetabolicArginine from the diet is absorbed into systemic circulation and enters hepatocytes through an amino acid transporter, where it undergoes a series of metabolic reactions. … If not cleared, it can reduce cardiomyocyte contractility and weaken cardiac efficiency, leading to systolic dysfunction and heart failure.
Enzymes: Y+L amino acid transporter 2Metabolism and Physiological Effects of Xanthine
smpdb.ca/view/SMP0124865MetabolicAdenosine 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. … The purines are guanine and adenine. These undergo metabolism in the liver to form xanthine. Adenine forms adenosine through the enzyme purine nucleoside phosphorylase.
Metabolism and Physiological Effects of Kynurenine
smpdb.ca/view/SMP0125524DiseaseIn the blood it travels to the liver and is transported into a hepatocyte by an amino acid transporter. … Tryptophan is consumed through foods such as milk, eggs, chicken, turkey, and oats. Tryptophan is then transported from the small intestine into the blood by an amino acid transport.
Enzymes: Neutral and basic amino acid transport protein rBAT, Neutral amino acid transporter AMetabolism 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. … and its isomer, pivaloylcarnitine; pivaloylcarnitine is derived from pivalate-generating antibiotics, and has caused many false-positive results.
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. … 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.