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Individuals with this disorder are known as "phenylketonurics" and must regulate their intake of phenylalanine. Phenylketonurics often use blood tests to monitor the amount of phenylalanine in their blood. Lab results may report phenylalanine levels using either mg/dL and μmol/L. One mg/dL of phenylalanine is approximately equivalent to 60 ...
Phenylalanine is a large, neutral amino acid (LNAA). LNAAs compete for transport across the blood–brain barrier (BBB) via the large neutral amino acid transporter (LNAAT). If phenylalanine is in excess in the blood, it will saturate the transporter. Excessive levels of phenylalanine tend to decrease the levels of other LNAAs in the brain.
Aspartame is a methyl ester of the dipeptide of the natural amino acids L - aspartic acid and L - phenylalanine. [ 4] Under strongly acidic or alkaline conditions, aspartame may generate methanol by hydrolysis. Under more severe conditions, the peptide bonds are also hydrolyzed, resulting in free amino acids.
Hyperphenylalaninemia is a medical condition characterized by mildly or strongly elevated concentrations of the amino acid phenylalanine in the blood. Phenylketonuria (PKU) can result in severe hyperphenylalaninemia. [2] Phenylalanine concentrations are routinely screened in newborns by the neonatal heel prick (Guthrie test), which takes a few ...
Aspartame, commonly known by the brand name NutraSweet, is one of the most commonly used artificial sweeteners. The 1982 introduction of aspartame-sweetened Diet Coke accelerated this trend. Today, at least in the United States, "diet" is nearly synonymous with the use of aspartame in beverages. Neotame and advantame are further derivatives of ...
Pegvaliase, sold under the brand name Palynziq, is a medication used for the treatment of the genetic disease phenylketonuria. [5] [9] [10] It is a phenylalanine (Phe)‑metabolizing enzyme. [5] Chemically, it is a pegylated derivative of the enzyme phenylalanine ammonia-lyase that metabolizes phenylalanine to reduce its blood levels.
A study of PKM2 in babies with the genetic brain disease phenylketonurics (PKU), showed elevated levels of phenylalanine and decreased effectiveness of PKM2. This inhibitory mechanism provides insight into the role of pyruvate kinase in brain cell damage.
List of naturally occurring phenethylamines. Trichocereus macrogonus var. pachanoi, syn. Echinopsis pachanoi contain several phenethylamines. Naturally occurring phenethylamines are organic compounds which may be thought of as being derived from phenethylamine itself that are found in living organisms. Tyramine is a phenethylamine that occurs ...