What type of protein is phenylalanine hydroxylase?
Phenylalanine hydroxylase (PAH) (EC 1.14. 16.1) is an enzyme that catalyzes the hydroxylation of the aromatic side-chain of phenylalanine to generate tyrosine.
What protein does the PAH gene code for?
phenylalanine hydroxylase
The PAH gene provides instructions for making an enzyme called phenylalanine hydroxylase. This enzyme is responsible for the first step in processing phenylalanine, which is a building block of proteins (an amino acid) obtained through the diet.
What proteins does phenylalanine produce?
Phenylalanine is also crucial for the production of other molecules, including (2): Tyrosine. This amino acid is produced directly from phenylalanine.
Is the PAH gene A protein?
PAH (Phenylalanine Hydroxylase) is a Protein Coding gene. Diseases associated with PAH include Phenylketonuria and Hyperphenylalaninemia. Among its related pathways are Peptide chain elongation and phenylalanine degradation/tyrosine biosynthesis.
What does Homogentisic acid oxidase do?
Homogentisate oxidase is responsible for a specific step in the breakdown of phenylalanine and tyrosine. Previous steps convert the two amino acids into a molecule called homogentisic acid. Homogentisate oxidase adds two oxygen atoms to homogentisic acid, converting it to another molecule called maleylacetoacetate.
What is the substrate for phenylalanine hydroxylase?
PAH is a member of the aromatic amino acid hydroxylase (AAAH) enzyme family. The AAAHs share a requirement for a catalytic non-heme ferrous iron, BH4 as cofactor, and molecular oxygen as additional substrate.
What is the chemical reaction catalyzed by phenylalanine hydroxylase?
Phenylalanine hydroxylase (PheH) catalyzes the hydroxylation of phenylalanine to tyrosine (Scheme 1) in catabolism of phenylalanine in the liver, with tetrahydrobiopterin (BH4) supplying the two electrons needed for the reaction (1).
What type of amino acid is phenylalanine?
essential amino acid
Phenylalanine is a type of amino acid. Amino acids are molecules that combine to form proteins. Phenylalanine is an essential amino acid in humans, meaning that the body cannot synthesize its own phenylalanine. Instead, humans must get phenylalanine from the foods they eat.
Why is phenylalanine an essential amino acid?
Phenylalanine is an essential amino acid meaning that it cannot be made in the body and must be ingested in the diet. Tyrosine is a nonessential amino acid and can be formed by the hydroxylation of phenylalanine in the liver when the intake of tyrosine in the diet is low.
Is phenylalanine an amino acid?
Phenylalanine is an amino acid, a “building block” of protein. There are three forms of phenylalanine: D-phenylalanine, L-phenylalanine, and the mix made in the laboratory called DL-phenylalanine.
How is homogentisic acid formed?
Homogentisic acid, also known as melanic acid, is an intermediate in the breakdown or catabolism of tyrosine and phenylalanine. It is generated from the compound p-hydroxyphenylpyruvate through the enzyme p-hydroxyphenylpyruvate dehydrogenase.
How is homogentisic acid formed in the body?
It is an intermediate in the catabolism of aromatic amino acids such as phenylalanine and tyrosine. 4-Hydroxyphenylpyruvate (produced by transamination of tyrosine) is acted upon by the enzyme 4-Hydroxyphenylpyruvate dioxygenase to yield homogentisate.