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Quinolacetic Acid
Quinolacetic acid is a chemical compound associated with the metabolic disorder hawkinsinuria. It is the byproduct of a partially defective enzyme, 4-hydroxyphenylpyruvate dioxygenase 4-Hydroxyphenylpyruvate dioxygenase (HPPD), also known as α-ketoisocaproate dioxygenase (KIC dioxygenase), is an Fe(II)-containing non-heme oxygenase that catalyzes the second reaction in the catabolism of tyrosine - the conversion of 4-hydroxy .... References {{organic-compound-stub Acetic acids Beta hydroxy acids Ketones ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hawkinsinuria
Hawkinsinuria is an autosomal dominant metabolic disorder affecting the metabolism of tyrosine. Normally, the breakdown of the amino acid tyrosine involves the conversion of 4-hydroxyphenylpyruvate to homogentisate by 4-hydroxyphenylpyruvate dioxygenase. Complete deficiency of this enzyme would lead to tyrosinemia III. In rare cases, however, the enzyme is still able to produce the reactive intermediate 1,2-epoxyphenyl acetic acid, but is unable to convert this intermediate to homogentisate. The intermediate then spontaneously reacts with glutathione to form 2-L-cystein-S-yl-1,4-dihydroxy-cyclohex-5-en-1-yl acetic acid (hawkinsin). Patients present with metabolic acidosis during the first year of life, and growth arrest around the time of weaning off breast milk. Treatment involves a diet containing a low amount of phenylalanine and tyrosine. Tolerance toward these amino acids normalizes as the patients get older. Then only a chlorine-like smell of the urine indicates the prese ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
4-hydroxyphenylpyruvate Dioxygenase
4-Hydroxyphenylpyruvate dioxygenase (HPPD), also known as α-ketoisocaproate dioxygenase (KIC dioxygenase), is an Fe(II)-containing non-heme oxygenase that catalyzes the second reaction in the catabolism of tyrosine - the conversion of 4-hydroxyphenylpyruvate into homogentisate. HPPD also catalyzes the conversion of phenylpyruvate to 2-hydroxyphenylacetate and the conversion of α-ketoisocaproate to β-hydroxy β-methylbutyrate. HPPD is an enzyme that is found in nearly all aerobic forms of life. Enzyme mechanism HPPD is categorized within a class of oxygenase enzymes that usually utilize α-ketoglutarate and diatomic oxygen to oxygenate or oxidize a target molecule. However, HPPD differs from most molecules in this class due to the fact that it does not use α-ketoglutarate, and it only utilizes two substrates while adding both atoms of diatomic oxygen into the product, homogentisate. The HPPD reaction occurs through a NIH shift and involves the oxidative decarboxylation ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Acetic Acids
Acetic acid , systematically named ethanoic acid , is an acidic, colourless liquid and organic compound with the chemical formula (also written as , , or ). Vinegar is at least 4% acetic acid by volume, making acetic acid the main component of vinegar apart from water and other trace elements. Acetic acid is the second simplest carboxylic acid (after formic acid). It is an important chemical reagent and industrial chemical, used primarily in the production of cellulose acetate for photographic film, polyvinyl acetate for wood glue, and synthetic fibres and fabrics. In households, diluted acetic acid is often used in descaling agents. In the food industry, acetic acid is controlled by the food additive code E260 as an acidity regulator and as a condiment. In biochemistry, the acetyl group, derived from acetic acid, is fundamental to all forms of life. When bound to coenzyme A, it is central to the metabolism of carbohydrates and fats. The global demand for acetic acid is ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Beta Hydroxy Acids
A beta hydroxy acid or β-hydroxy acid (BHA) is an organic compound that contains a carboxylic acid functional group and hydroxy functional group separated by ''two'' carbon atoms. They are closely related to alpha hydroxy acids, in which the two functional groups are separated by ''one'' carbon atom. In cosmetics, the term ''beta hydroxy acid'' refers specifically to salicylic acid, which is used in some "anti-aging" creams and acne treatments. It is used to combat inflammation. Upon dehydration, beta-hydroxy acids yield an alpha-beta unsaturated acid. Acidic properties Compared to non-hydroxylated carboxylic acids, this group of acids is stronger, although less strong than the alpha hydroxy acids. Due to the larger distance, the intramolecular hydrogen bridge is less easily formed compared to the alpha hydroxy acids. The table summarizes some values on the propionic series. {, class="wikitable" ! Name ! p''K''a , - , Propanoic acid , , 4.87Handbook of Chemistry ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
