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Valine, Leucine, and Isoleucine Degradation
smpdb.ca/view/SMP0000032MetabolicValine, isoleuciine, and leucine are essential amino acids and are identified as the branched-chain amino acids (BCAAs). … The catabolism of all three amino acids starts in muscle and yields NADH and FADH2 which can be utilized for ATP generation.
Enzymes: Branched-chain-amino-acid aminotransferase, cytosolic, Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
smpdb.ca/view/SMP0000521DiseaseThis 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. … acid.
Enzymes: Branched-chain-amino-acid aminotransferase, cytosolic, Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrialMetabolism and Physiological Effects of Dimethylglycine
smpdb.ca/view/SMP0124873MetabolicDimethylglycine (DMG) is an amino acid derivative found in the cells of all plants and animals and can be obtained in the diet in small amounts from grains and meat. … Dimethylglycine enters the bloodstream and can cause toxic effects on the cardiovascular system.
Drugs: Choline · Methionine · NADH · Zinc · N,N-dimethylglycine · Flavin adenine dinucleotide +1 moreCongenital 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
Intracellular Signalling Through Prostacyclin Receptor and Prostacyclin
smpdb.ca/view/SMP0000354SignalingProstacyclin binding to its receptor increases vasodilation and decreases platelet aggregation. … The receptor is a G-protein coupled receptor, upon its binding it activates G proteins causing the activation of adenylyl cyclase and production of cAMP messenger molecules. cAMP activates PKA (protein
Metabolsim and Physiological Effects of 4-Ethylphenylsulfate
smpdb.ca/view/SMP0125541Metabolicacid. … This is metabolized into phenylpyruvic acid Phenylacetic acid which is then metabolized into 3-(3-Hydroxyphenyl)propanoic acid.
Drugs: Pyridoxal phosphate · Phenylalanine · Tyrosine · Iron · Oxogluric acid · Propanoic acid +3 moreEnzymes: Aromatic-amino-acid aminotransferaseCongenital Bile Acid Synthesis Defect Type II
smpdb.ca/view/SMP0000314DiseaseChenodeoxycholic acid and cholic acid are decreased in plasma and urine. … Symptoms of this disease include cholestatic jaundice, atypical oxo and allo bile acids in urine and serum, liver failure, and steatosis.
Congenital Bile Acid Synthesis Defect Type III
smpdb.ca/view/SMP0000318DiseaseCBASIII 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
Congenital Bile Acid Synthesis Defect Type II
smpdb.ca/view/SMP0125689DiseaseChenodeoxycholic acid and cholic acid are decreased in plasma and urine. … Symptoms of this disease include cholestatic jaundice, atypical oxo and allo bile acids in urine and serum, liver failure, and steatosis.
Metabolism and Physiological Effects of O-Sulfotyrosine
smpdb.ca/view/SMP0126811MetabolicO-Sulfotyrosine belongs to the class of organic compounds known as phenylalanine and derivatives. … Secreted proteins and extracellular parts of membrane proteins that pass through the Golgi apparatus may be sulfated.