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Fumarylacetoacetate
Fumarylacetoacetic acid (fumarylacetoacetate) is an intermediate in the metabolism of tyrosine. It is formed through the conversion of maleylacetoacetate into fumarylacetoacetate by the enzyme maleylacetoacetate isomerase. See also * Fumarylacetoacetate hydrolase Fumarylacetoacetase is an enzyme that in humans is encoded by the ''FAH'' gene located on chromosome 15. The FAH gene is thought to be involved in the catabolism of the amino acid phenylalanine in humans. Function Fumarylacetoacetate hydrolase ( ... References Dicarboxylic acids Beta-keto acids Enones {{biochem-stub ...
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Fumarylacetoacetate Hydrolase
Fumarylacetoacetase is an enzyme that in humans is encoded by the ''FAH'' gene located on chromosome 15. The FAH gene is thought to be involved in the catabolism of the amino acid phenylalanine in humans. Function Fumarylacetoacetate hydrolase (FAH) is a protein homodimer which cleaves fumarylacetoacetate at its carbon-carbon bond during a hydrolysis reaction. As a critical enzyme in phenylalanine and tyrosine metabolism,  4-Fumarylacetoacetate hydrolase catalyzes the final step in the catabolism of 4-fumarylacetoacetate and water into acetoacetate, fumarate, and H+ respectively. These hydrolytic reactions are essential during aromatic amino acid human metabolism. Furthermore, FAH does not share known protein sequence homologs with other nucleotides or amino acids. Reaction mechanism The active site of FAH contains  Ca2+ which acts to bind the substrate and a Glu-His-Water catalytic triad functions where  the imidaxole ring of His133 activates a nucleophilic water molecule ...
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Tyrosine
-Tyrosine or tyrosine (symbol Tyr or Y) or 4-hydroxyphenylalanine is one of the 20 standard amino acids that are used by cells to synthesize proteins. It is a non-essential amino acid with a polar side group. The word "tyrosine" is from the Greek ''tyrós'', meaning ''cheese'', as it was first discovered in 1846 by German chemist Justus von Liebig in the protein casein from cheese. It is called tyrosyl when referred to as a functional group or side chain. While tyrosine is generally classified as a Hydrophobe, hydrophobic amino acid, it is more hydrophilic than phenylalanine. It is Genetic code, encoded by the Genetic code#Codons, codons UAC and UAU in messenger RNA. Functions Aside from being a proteinogenic amino acid, tyrosine has a special role by virtue of the phenol functionality. It occurs in proteins that are part of signal transduction processes and functions as a receiver of phosphate groups that are transferred by way of protein kinases. Phosphorylation of the hyd ...
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Maleylacetoacetate
4-Maleylacetoacetate (4-maleylacetoacetatic acid) is an intermediate in the metabolism of tyrosine. It is converted to fumarylacetoacetate by the enzyme 4-maleylacetoacetate cis-trans-isomerase. Gluthathione coenzymatically helps in conversion to fumarylacetoacetic acid. See also * Homogentisate 1,2-dioxygenase Homogentisate 1,2-dioxygenase (homogentisic acid oxidase, homogentisate oxidase, homogentisicase) is an enzyme which catalyzes the conversion of homogentisate to 4-Maleylacetoacetate, 4-maleylacetoacetate. Homogentisate 1,2-dioxygenase or HGD ... Beta-keto acids Enones Diketones {{biochem-stub ...
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Maleylacetoacetate Isomerase
In enzymology, maleylacetoacetate isomerase () is an enzyme that catalyzes the chemical reaction :4-maleylacetoacetate \rightleftharpoons 4-fumarylacetoacetate This enzyme belongs to the family of isomerases, specifically ''cis''-''trans'' isomerases. The systematic name of this enzyme class is 4-maleylacetoacetate ''cis''-''trans''-isomerase. 4-Maleylacetoacetate isomerase is an enzyme involved in the degradation of L-phenylalanine. It is encoded by the gene glutathione S-transferase zeta 1, or GSTZ1. This enzyme catalyzes the conversion of 4-maleylacetoacetate to 4-fumarylacetoacetate. 4-Maleylacetoacetate isomerase belongs to the zeta class of the glutathione S-transferase (GST) superfamily.; Mechanism In the  phenylalanine degradation pathway, 4-maleylacetoacetate isomerase catalyzes a ''cis''-''trans'' isomerization of  4-maleylacetoacetate to fumarylacetoacetate. 4-maleylacetoacetate isomerase requires the cofactor glutathione to function. Ser 15, Cys 16, Gln 1 ...
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Dicarboxylic Acids
In organic chemistry, a dicarboxylic acid is an organic compound containing two carboxyl groups (). The general molecular formula for dicarboxylic acids can be written as , where R can be aliphatic or aromatic. In general, dicarboxylic acids show similar chemical behavior and reactivity to monocarboxylic acids. Dicarboxylic acids are used in the preparation of copolymers such as polyamides and polyesters. The most widely used dicarboxylic acid in the industry is adipic acid, which is a precursor in the production of nylon. Other examples of dicarboxylic acids include aspartic acid and glutamic acid, two amino acids in the human body. The name can be abbreviated to diacid. Linear saturated dicarboxylic acids The general formula is .Boy Cornils, Peter Lappe "Dicarboxylic Acids, Aliphatic" in Ullmann's Encyclopedia of Industrial Chemistry 2014, Wiley-VCH, Weinheim. The PubChem links gives access to more information on the compounds, including other names, ids, toxicity and sa ...
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