A method for phenylalanine self-monitoring using phenylalanine ammonia-lyase and a pre-existing portable ammonia detection system

Wada, Yoichi; Totsune, Eriko; Mikami-Saito, Yasuko; Kikuchi, Atsuo; Miyata, Toshio; Kure, Shigeo

doi:10.1016/j.ymgmr.2023.100970

Cited by 4 publications

(4 citation statements)

References 39 publications

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“…L-Glutamine can reduce the incidence of vascular occlusion crisis (VOC) [ 37 ]. Phenylalanine is an indispensable substance for patients with phenylketonuria [ 38 ].…”

Section: Resultsmentioning

confidence: 99%

Revealing Medicinal Constituents of Bistorta vivipara Based on Non-Targeted Metabolomics and 16S rDNA Gene Sequencing Technology

He,

Tang,

Cao

et al. 2024

Molecules

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Bistorta vivipara is a medicinal plant with a long history, but there are few studies on the effects of its medicinal components and endophytic bacteria on the accumulation of secondary metabolites. Therefore, in this study, non-targeted metabolomics techniques and 16s rDNA techniques were used to study B. vivipara from different regions. A total of 1290 metabolites and 437 differential metabolites were identified from all samples. Among them, flavonoids, isoflavonoids, and benzopyrans are the main medicinal components of B. vivipara; these have potential anticancer, antiviral, and antioxidant properties, as well as potential applications for the treatment of atrial fibrillation. In addition, irigenin, an important medicinal component, was identified for the first time. The endophytic bacterial communities in the root tissues of B. vivipara from different regions were also different in composition and richness. Hierarchical clustering heat map analysis showed that Proteobacteria and Actinobacteriota bacteria significantly affected the accumulation of many medicinal components in the roots of B. vivipara.

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“…L-Glutamine can reduce the incidence of vascular occlusion crisis (VOC) [ 37 ]. Phenylalanine is an indispensable substance for patients with phenylketonuria [ 38 ].…”

Section: Resultsmentioning

confidence: 99%

Revealing Medicinal Constituents of Bistorta vivipara Based on Non-Targeted Metabolomics and 16S rDNA Gene Sequencing Technology

He,

Tang,

Cao

et al. 2024

Molecules

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“…Compared with diabetic patients who can easily self-monitor their insulin levels, patients with PKU rely on the results of submitted blood spots that can take 1–2 weeks to receive, contributing to the treatment burden. Currently, home Phe-monitoring systems are still in development [ 101 ]. When available, such a system may increase patient self-empowerment regardless of therapeutic management [ 100 , 101 ].…”

Section: Resultsmentioning

confidence: 99%

“…Currently, home Phe-monitoring systems are still in development [ 101 ]. When available, such a system may increase patient self-empowerment regardless of therapeutic management [ 100 , 101 ].…”

Section: Resultsmentioning

confidence: 99%

Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria

Rocha,

Ahring,

Bausell

et al. 2023

Nutrients

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Many adults with phenylketonuria (PKU) rely on medical nutrition therapy (MNT; low phenylalanine (Phe) diet with protein substitutes/medical foods) to maintain blood Phe concentrations within recommended ranges and prevent PKU-associated comorbidities. Despite disease detection through newborn screening and introduction of MNT as early as birth, adherence to MNT often deteriorates from childhood onwards, complicating the assessment of its effectiveness in the long term. Via a modified Delphi process, consensus (≥70% agreement) was sought on 19 statements among an international, multidisciplinary 13-member expert panel. After three iterative voting rounds, the panel achieved consensus on 17 statements related to the limitations of the long-term effectiveness of MNT (7), the burden of long-term reliance on MNT (4), and its potential long-term detrimental health effects (6). According to the expert panel, the effectiveness of MNT is limited in the long term, is associated with a high treatment burden, and demonstrates that adults with PKU are often unable to achieve metabolic control through dietary management alone, creating an unmet need in the adult PKU population.

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“…During the last years, different enzymatic and nonenzymatic approaches for Phe recognition have been reported in the literature. Most of them are based on colorimetric − and electric detection, − and some of these were successfully integrated into miniaturized devices, , including wearable sensors . Despite the declared good analytical performances, they appear difficult to be developed in a fully integrated platform for commercial purposes, due to different constraints such as high cost analysis, laborious procedures, and limited usability.…”

mentioning

confidence: 99%

Fully Integrated Point-of-Care Platform for the Self-Monitoring of Phenylalanine in Finger-Prick Blood

Messina,

Maugeri,

Spoto

et al. 2023

ACS Sens.

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Development of point-of-care platforms combining reliability and ease of use is a challenge for the evolution of sensing in healthcare technologies. Here, we report the development and testing of a fully integrated enzymatic colorimetric assay for the sensing of phenylalanine in blood samples from phenylketonuria patients. The platform works with a customized mobile app for data acquisition and visualization and comprises an electronic system and a disposable sensor. The sensing approach is based on specific enzymatic phenylalanine recognition, and the optical transduction method is based on in situ gold nanostructure formation. The phenylketonuria (PKU) smart sensor platform is conceived to perform self-monitoring on phenylalanine levels and real-time therapy tuning, thanks to the direct connection with clinicians. Validation of the technologies with a population of patients affected by PKU, together with the concurrent validation of the platform through centralized laboratories, has confirmed the good analytical performances in terms of sensitivity and specificity, robustness, and utility for phenylalanine sensing. The self-monitoring of phenylalanine for the daily identification of abnormal health conditions could facilitate rapid therapy tuning, improving the wellness of PKU patients.

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A method for phenylalanine self-monitoring using phenylalanine ammonia-lyase and a pre-existing portable ammonia detection system

Cited by 4 publications

References 39 publications

Revealing Medicinal Constituents of Bistorta vivipara Based on Non-Targeted Metabolomics and 16S rDNA Gene Sequencing Technology

Revealing Medicinal Constituents of Bistorta vivipara Based on Non-Targeted Metabolomics and 16S rDNA Gene Sequencing Technology

Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria

Fully Integrated Point-of-Care Platform for the Self-Monitoring of Phenylalanine in Finger-Prick Blood

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