Tryptophan metabolism: utility of plasmatic assay in phenylketonuria, a study in 6 adult patients

Boulet, Lysiane; Besson, Gérard; Faure, Patrice; Ducros, Véronique; Corne, Christelle

doi:10.1684/abc.2018.1334

Cited by 2 publications

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“…For all these reasons, we hypothetized a possible modification in Trp pathway in adult PKU population, which could be linked to some neurological perturbations. A preliminary study showed significant decreased of Trp, KYN, and 3HK blood concentrations in six adult PKU patients 18 . In the present study, we evaluated blood Trp metabolites levels in a large cohort of adults with PKU (from French ECOPHEN study).…”

Section: Introductionmentioning

confidence: 98%

“…A preliminary study showed significant decreased of Trp, KYN, and 3HK blood concentrations in six adult PKU patients. 18 In the present study, we evaluated blood Trp metabolites levels in a large cohort of adults with PKU (from French ECOPHEN study). PKU patients were classified in three groups: patients with no specific diet, patients with only controlled low-Phe diet, and patients with controlled low-Phe diet and taking AA substitute.…”

Section: Introductionmentioning

confidence: 99%

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Tryptophan metabolism in phenylketonuria: A French adult cohort study

Boulet

Besson

Noolen

et al. 2020

J of Inher Metab Disea

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Many similarities between tryptophan (Trp) and phenylalanine (Phe) metabolisms exist. It is possible that a modification of Trp metabolism might be seen in phenylketonuria (PKU). As some of these metabolites have neuroactive properties, they should be consider in neurological impairment seen in this pathology and not totally explained by blood Phe concentrations. One hundred and fifty‐one adult PKU patients (mean age 26.8 years) were included for this study. Plasma Trp, kynurenine (KYN), 3‐hydroxykynurenic acid (3HK), and kynurenic acid (KA) were analyzed by liquid chromatography coupled with tandem mass spectrometry. KYN and 3HK were significantly lower in PKU patients compared to general population (P < .0001), and KA was significantly enhanced is this population (P = .009). Furthermore, 3HK concentration was significantly different between PKU patients underwent controlled low‐Phe diet compared to PKU patients without this diet (P = .0016). In PKU patients with diet, taking AA substitute enable higher plasma 3HK concentration than without (P = .0008) but still not reaching general population level (P < .0001). Although further study has to be done, it is clear that Trp metabolism is modified in adult PKU patients. An exploration of complete Trp metabolism, and not only Trp concentration, is needed in PKU population, but also in other inborn error of metabolism treated with hypoprotidic diet.

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Section: Introductionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

Tryptophan metabolism in phenylketonuria: A French adult cohort study

Boulet

Besson

Noolen

et al. 2020

J of Inher Metab Disea

Self Cite

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Rat PKU Model Display Gender-Based Neuroinflammatory Changes: Proinflamatuary Cytokines and Lipid Peroxidation

Çiçek,

Gök,

Bodur

2024

Muğla Sıtkı Koçman Üniversitesi Tıp Dergisi

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Phenylketonuria (PKU) results from congenital defects of amino acid metabolism. Accumulated phenylalanine crosses the blood-brain barrier and causes permanent brain damage, but the neuro-pathophysiology underlying phenylketonuria is not fully understood. Chemically-induced rat phenylketonuria model of both genders was generated to examine the role of inflammatory response, lipid peroxidation and oxidative stress in the prefrontal cortex. Our results showed that in phenylketonuria there was an increase in lipid peroxidation compared to controls, which was significantly different only in males (p

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Tryptophan metabolism: utility of plasmatic assay in phenylketonuria, a study in 6 adult patients

Cited by 2 publications

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Tryptophan metabolism in phenylketonuria: A French adult cohort study

Tryptophan metabolism in phenylketonuria: A French adult cohort study

Rat PKU Model Display Gender-Based Neuroinflammatory Changes: Proinflamatuary Cytokines and Lipid Peroxidation

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