What does Homogentisate oxidase do?

What does Homogentisate 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.

Why is Homogentisate oxidase called dioxygenase enzyme?

Homogentisate 1,2-dioxygenase or HGD is involved in the catabolism of aromatic rings, more specifically in the breakdown of the amino acids tyrosine and phenylalanine. HGD appears in the metabolic pathway of tyrosine and phenylalanine degradation once the molecule homogentisate is produced.

What is an HGD?

HGD: The gene encoding an enzyme called homogentisate 1,2-dioxygenase (also known as homogentisate oxidase). HGD is located on chromosome 3 in region 3q21-q23. Homogentisate oxidase is active chiefly in the liver and kidneys. It is needed to break down the amino acids phenylalanine and tyrosine.

Where does homogentisic acid come from?

Homogentisic acid (2,5-dihydroxyphenylacetic acid) is a phenolic acid usually found in Arbutus unedo (strawberry-tree) honey. It is also present in the bacterial plant pathogen Xanthomonas campestris pv. phaseoli as well as in the yeast Yarrowia lipolytica where it is associated with the production of brown pigments.

What is the function of homogentisic acid?

When present in sufficiently high levels, homogentisic acid can function as an osteotoxin and a renal toxin. An osteotoxin is a substance that causes damage to bones and/or joints. A renal toxin causes damage to the kidneys.

What do Dioxygenases do?

Dioxygenases catalyze the incorporation of both atoms of O2 into substrates. When both oxygen atoms are incorporated into the same molecule, it is referred to as an intramolecular dioxygenase; when the two atoms of oxygen are incorporated into two products, it is an intermolecular dioxygenase.

How is Alkapton formed?

Alkaptonuria is an autosomal recessive disorder caused by a deficiency of the enzyme homogentisate 1,2-dioxygenase. This enzyme deficiency results in increased levels of homogentisic acid, a product of tyrosine and phenylalanine metabolism.

What causes high homogentisic acid?

Alkaptonuria is a rare inherited disorder. It occurs when your body can’t produce enough of an enzyme called homogentisic dioxygenase (HGD). This enzyme is used to break down a toxic substance called homogentisic acid. When you don’t produce enough HGD, homogentisic acid builds up in your body.

What is the role of homogentisate 1, 2 dioxygenase?

Homogentisate 1,2-dioxygenase ( homogentisic acid oxidase, homogentisicase) is an enzyme which catalyzes the conversion of homogentisate to 4-maleylacetoacetate. Homogentisate 1,2-dioxygenase or HGD is involved in the catabolism of aromatic rings, more specifically in the breakdown of…

What happens when HGD reacts with homogentisate?

HGD appears in the metabolic pathway of tyrosine and phenylalanine degradation once the molecule homogentisate is produced. Homogentisate reacts with HGD to produce maleylacetoacetate, which then is further used in the metabolic pathway. HGD requires the use of Fe 2+ and O 2 in order to cleave the aromatic ring of homogentisate.

Which is the catalysis of homogentisate ( HPPD )?

4-Hydroxyphenylpyruvate dioxygenase (HPPD) catalyzes the conversion of 4-hydroxyphenylpyruvate (HPP) into homogentisate. HPPD is the molecular target of very effective synthetic herbicides. HPPD inhibitors may also be useful in treating life-threatening tyrosinemia type I and are currently in trials for treatment of Parkinson disease.

What kind of enzyme is HGD 1, 2 dioxygenase?

HGD homogentisate 1,2-dioxygenase [ (human)] Summary. This gene encodes the enzyme homogentisate 1,2 dioxygenase. This enzyme is involved in the catabolism of the amino acids tyrosine and phenylalanine.