4-Hydroxyphenylpyruvate

4-Hydroxyphenylpyruvate is an intermediate in the metabolism of the amino acid phenylalanine. It is converted into homogentisate by the enzyme 4-hydroxyphenylpyruvate dioxygenase.

Structure and properties

4-Hydroxyphenylpyruvate is a ketone and a hydroxy acid. It is derived from a phenylalanine. It is a conjugate acid of a 4-hydroxyphenylpyruvate(1-).

Role in metabolism

In humans, 4-Hydroxyphenylpyruvate is a key intermediate in the catabolic pathway of phenylalanine and tyrosine, two of the twenty standard amino acids used to synthesize proteins. This metabolic pathway is responsible for the degradation of these amino acids into fumarate and acetoacetate, which can be used to produce energy in the form of adenosine triphosphate (ATP).

Clinical significance

Deficiency in the enzyme 4-hydroxyphenylpyruvate dioxygenase, which catalyzes the conversion of 4-Hydroxyphenylpyruvate to homogentisate, can lead to Type III tyrosinemia, a rare inherited disorder characterized by elevated levels of tyrosine in the blood and urine.

See also

• Phenylalanine
• Tyrosine
• 4-hydroxyphenylpyruvate dioxygenase
• Type III tyrosinemia

4-Hydroxyphenylpyruvate Resources
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