Phenylalanine is an amino acid that must be obtained from the diet. It is needed to make tyrosine, an important substance from which the skin pigment melanin, the thyroid hormone thyroxine, and the neurotransmitters catecholamines are made. It is also degraded by conversion to homogentisic acid. The normal phenylalanine-tyrosine metabolic pathway is as follows: Phenylalanine … Disorders of Phenylalanine-Tyrosine Metabolism
Products Most HIV protease inhibitors are commercially available in tablet or capsule form. In addition, a few liquid dosage forms are available for ingestion. Saquinavir (Invirase) was the first to be lanicized in 1995. Structure and properties The first HIV protease inhibitors were modeled on the natural peptide substrate of the HIV protease. The protease … HIV Protease Inhibitor
In 1963, the American physician and microbiologist Robert Guthrie introduced the dry blood test, the Guthrie test, with which he was able to diagnose the metabolic disorder phenylkenonuria (inability to break down the amino acid phenylalanine due to the lack of an important enzyme in the body) in newborns. Even today, this screening method is … Dry Blood Test: Treatment, Effect & Risks
Products No drugs containing chloramphenicol for systemic use are commercially available in many countries. Structure and properties Chloramphenicol (C11H12Cl2N2O5, Mr = 323.1 g/mol) is a white to grayish white or yellowish white, fine, crystalline powder or exists as fine, acicular or elongated flat crystals. It is sparingly soluble in water. Chloramphenicol is formed during the … Chloramphenicol Effects and Side Effects
Products Nitrogen is commercially available as a compressed gas in pressurized cylinders and as a liquid in cryogenic containers, among other products. Structure and properties Nitrogen (N, atomic mass: 14.0 u) is a colorless and odorless gas that is present in over 78% of air. It is a chemical element with atomic number 7 and … Nitrogen
Background Phenylalanine is an essential amino acid that is not produced by the human organism itself. Phenylalanine ingested with food is produced by the enzyme phenylalanine hydroxylase and its cofactor 6-tetrahydrobiopterin (6-BH4) metabolized to tyrosine. Phenylketonuria is an autosomal recessive disorder caused by insufficient activity of phenylalanine hydroxylase, resulting in elevated blood phenylalanine levels, i.e., … Sapropterin
Products Epinephrine is commercially available as an injection solution and as an epinephrine auto-injector from various suppliers. The active ingredient is also known as epinephrine, especially in English (in German: Epinephrin). Structure and properties Epinephrine (C9H13NO3, Mr = 183.2 g/mol) exists as a white, crystalline powder with a bitter taste that turns brownish on contact … Adrenaline
Products Afamelanotide is administered as an implant (Scenesse, Clinuvel). It has had orphan drug status in many countries since 2008. It has not yet been registered with Swissmedic and is not approved as a drug. In the United States, the drug was approved in 2019. Structure and properties Afamelanotide is an analog of α-melanocyte-stimulating hormone … Afamelanotide
Definition – What is phenylketonuria? Phenylketonuria is a hereditary disease pattern that is expressed in a reduced breakdown of the amino acid phenylalanine. The tricky thing about the disease is that it has been present since birth and thus leads to an accumulation of the amino acid. From about the third month of life it … Phenylketonuria
Diagnosis of Phenylketonuria Diagnosis is carried out in two different ways as standard. One is the detection of the defective enzyme, the other is the detection of the greatly increased phenylalanine concentration in the blood. The first method is part of newborn screening as so-called tandem mass spectroscopy and indicates the defect without the need … Diagnosis of Phenylketonuria | Phenylketonuria
Prognosis vs. life expectancy in phenylketonuria The life expectancy of the affected persons depends on the one hand on the present form of phenylketonuria and on the other hand on the time when the disease is diagnosed. While a normal life expectancy is possible with a normal variant of phenylketonuria, there are rare variants of … Prognosis vs. life expectancy in phenylketonuria | Phenylketonuria
Amino acids are the basic substances of proteins and there are 20 different amino acids from which the body can form many different proteins among other substances. The 20 amino acids can be divided into two groups, the essential and the non-essential amino acids. There are eight essential amino acids, isoleucine, leucine, lysine, methionine, phenylalanine, … Amino acids list
