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PKU Talks: The expert’s voice

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“PKU TALKS: THE EXPERT’S VOICE” is the periodical column on Phenylketonuria, created by Piam in collaboration with some of the major international specialists.

Today an interview with Dr. Francesco Porta, pediatrician medical director at the Hospital Regina Margherita di Torino,about the role of tyrosine as a marker for monitoring Phenylketonuria.

What is the role of tyrosine for monitoring disease in PKU patients?

Historically, in the PKU field, researchers have always studied the substrate phenylalanine, which is toxic for the brain. It is important to remember that tyrosine is not only a product of phenylalanine hydroxylation, but also a substrate for other molecules such as hormones and neurotransmitters. So, the metabolic monitoring of tyrosine in PKU may have a really important role for the follow up of the PKU patient.

Besides and together with phenylalanine, could tyrosine be a valuable marker for monitoring the management of PKU?

Tyrosine may be a new marker to be followed up in the biochemical monitoring of the PKU patients. Also the phenylalanine/tyrosine ratio may become a very important marker for the follow up of PKU patients.