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Lysosomal Acid Lipase Deficiency (Wolman Disease)
smpdb.ca/view/SMP0000319DiseaseLysosomal 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 hydrolaseIndocyanine green acid form Drug Metabolism
smpdb.ca/view/SMP0130870MetabolicIndocyanine green acid form passes through the liver and is then excreted from the body mainly through the kidney. … Indocyanine green acid form is a drug that is not metabolized by the human body as determined by current research and biotransformer analysis.
Drugs: Indocyanine green acid formApoptotic DNA Fragmentation and Tissue Homeostasis
smpdb.ca/view/SMP0063772SignalingICAD and CAD exist in the nucleus in normal cells and is a major endonuclease in apoptosis. Its activation is normally caspase dependent. … EndoG resides in mitochondria in normal cells and travels to the nucleus, where it fragments chromosomal DNA upon activation of apoptosis.
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. … First, it forms guanidinoacetic acid in a reaction catalysed by glycine amidinotransferase, then undergoes a reaction catalysed by guanidinoacetate N-methyltransferase to form creatine.
Enzymes: Y+L amino acid transporter 2Metabolism and Physiological Effects of Xanthine
smpdb.ca/view/SMP0124865MetabolicThe purines are guanine and adenine. These undergo metabolism in the liver to form xanthine. Adenine forms adenosine through the enzyme purine nucleoside phosphorylase. … Adenosine 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.
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/SMP0126748MetabolicIsovalerylglycine (IVG) is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. … In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation.
Metabolism and Physiological Effects of Tiglylglycine
smpdb.ca/view/SMP0126840MetabolicTiglylglycine is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. … In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation.
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 is also produced as a byproduct of the enzymatic production of nitric oxide from the amino acid arginine, catalyzed by nitric oxide synthase.
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