Beneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria

Scala, Iris; Concolino, Daniela; Nastasi, A; Esposito, Giulia; Crisci, Daniela; Sestito, Simona; Ferraro, Stefania; Albano, Lucia; Ruoppolo, Margherita; Parenti, Giancarlo; Strisciuglio, Pietro

doi:10.3390/nu13114012

Cited by 3 publications

(2 citation statements)

References 44 publications

(61 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Controlled release systems over coated amino acids are also desired for people with inborn errors in amino acid metabolism e.g. phenylketonuria 19 – 21 .…”

Section: Introductionmentioning

confidence: 99%

Synthesis of L-methionine-loaded chitosan nanoparticles for controlled release and their in vitro and in vivo evaluation

Nuzaiba

Gupta

Gupta³

et al. 2023

Sci Rep

View full text Add to dashboard Cite

Therapeutically popular controlled release-enabling technology has forayed into the nutrition sector. Polymer coated forms of L-methionine used in soy protein diets, and its intermediate metabolite, S-adenosyl-L-methionine, used in myriad of medical conditions have proved more efficacious over (highly catabolized) free forms. In this premier study, L-methionine-loaded chitosan nanoparticles (M-NPs) were synthesized using ionic gelation method and their efficacy was evaluated. Biophysical characterization of the NPs was done using a Nanopartica SZ 100 analyser, transmission electron microscopy, and Fourier transform infrared spectroscopy. The M-NPs were spherical and smooth and 218.9 ± 7.4 nm in size and in vitro testing confirmed the controlled release of methionine. A 60-days feeding trial in L. rohita fish fingerlings was conducted. A basal diet suboptimal (0.85%) in methionine was provided with one of the supplements as under: none (control), 0.8% chitosan NPs (0.8% NPs), 1.2% L-methionine (1.2% M) (crystalline free form), 0.6% M-NPs and 1.2% M-NPs. While the addition of 0.6% M-NPs to the basal diet complemented towards meeting the established dietary requirement and resulted in significantly highest (P < 0.05) growth and protein efficiency and sero-immunological test scores (serum total protein, serum globulin, serum albumin: globulin ratio, phagocytic respiratory burst/NBT reduction and lysozyme activity), 1.2% supplementation in either form (free or nano), for being 0.85% excess, was counterproductive. Liver transaminases and dehydrogenases corroborated enhanced growth. It was inferred that part of the methionine requirement in nano form (M-NPs) can confer intended performance and health benefits in animals relying on plant proteins-based diets limiting in this essential amino acid. The study also paves the way for exploring chitosan NPs-based sustained delivery of amino acids in human medical conditions.

show abstract

“…Controlled release systems over coated amino acids are also desired for people with inborn errors in amino acid metabolism e.g. phenylketonuria 19 – 21 .…”

Section: Introductionmentioning

confidence: 99%

Synthesis of L-methionine-loaded chitosan nanoparticles for controlled release and their in vitro and in vivo evaluation

Nuzaiba

Gupta

Gupta³

et al. 2023

Sci Rep

View full text Add to dashboard Cite

show abstract

“…The SLC7A8 gene encoding LAT2 is located at 14q11.2 and like LAT1, LAT2 is associated with the glycoprotein 4F2hc, and their sequences are in 50% homogenous [4]. LAT2 is mainly expressed in the kidney, jejunum, ileum, lung, heart, and spleen [12]. In contrast to LAT1, LAT2 transports thyroid hormones only inwards and the transport of the drug via BBB is negligible [13].…”

Section: Introductionmentioning

confidence: 99%

Transcriptomic data analysis of melanocytes and melanoma cell lines of LAT transporter genes for precise medicine

Szczepanek

Panek

Przybyło

et al. 2022

Bio-Algorithms and Med-Systems

View full text Add to dashboard Cite

Background: Boron Neutron Capture Therapy (BNCT) is a two-step treatment that can be used in some types of cancers. It involves administering a compound containing boron atoms to the patient and irradiating the affected area of the body with a neutron beam. The success of the therapy depends mainly on the delivery of the boron isotope (10B) to the tumor using an appropriate boron carrier. One of the boron carriers used is boronophenylalanine (BPA). Therefore, in research on the use of boron carriers, it is also important to know the mechanisms of its uptake by cells. Aim: To study the expression of LAT family genes in two melanoma (high melanotic WM115 and low melanotic WM266-4) cell lines and melanocytes (HEMa-Lp) which are responsible for the transport the BPA into cells. Methods: To normalize data from the transcriptomic analysis, the ratio of the median method was used. This allowed the samples to be compared with each other. Comparison metrics included log-fold change (LFC) values. The heatmap of LFC values and the cluster map were created. These graphs show the similarities and differences between the samples. Results: Transcriptomic data show that in melanocytes, LFC for SLC7A5 (LAT1) and SLC3A2 (4Fhc) was higher than in melanoma cell lines, which corresponded with their melanin content. Conclusion: Our results indicate overexpression of BPA transporter genes in normal cells (melanocytes), which may suggest the highest level of these proteins in melanocytes compared to less melanotic melanoma. Therefore, for BNCT, the use of BPA as the 10B carrier will require additional qualifying tests of amino acid transporter expression for patients and specific tumors to develop a personalized BNCT.

show abstract

Benefits of a prolonged-release amino acid mixture in four pregnant women with phenylketonuria

Sestito,

Brodosi,

Ferraro

et al. 2024

Nutr Health

View full text Add to dashboard Cite

Background: Maternal phenylketonuria (mPKU) is a pathologic condition occurring in the fetus of a mother with PKU that is caused by prolonged elevated intrauterine blood phenylalanine (Phe) levels, which can lead to congenital abnormalities and mental retardation of newborns. Management of PKU during pregnancy can be challenging as protein substitutes may exacerbate nausea, vomiting, and gastrointestinal symptoms. Aim: To report the successful management of four PKU pregnant women. Methods: The patients were administered with prolonged-release amino acid supplementation and were recommended to follow a strict diet. Blood Phe concentration, adherence to diet, and occurrence of adverse events were monitored. Results: All patients achieved safe levels of blood Phe concentration (120–360 µmol/L) since preconception and during pregnancy (mean Phe concentration values of 143.34 ± 137.59, 226.48 ± 194.57, 186.68 ± 133.67, and 187.47 ± 42.59 µmol/L). During the first trimester of pregnancy, all patients manifested gastrointestinal symptoms such as nausea, gastrointestinal reflux, and abdominal bloating, which were managed by either changing protein substitute or extending the time window between different meals and amino acid mixtures administration. The four women continued their pregnancies without experiencing further complications and delivered neonates with normal growth parameters and no malformations. Conclusion: Findings of this case series suggest that the intake of a prolonged-release amino acid mixture in granules is well tolerated by pregnant PKU patients, eventually leading to good metabolic control and fetal growth within normal ranges.

show abstract

Beneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria

Cited by 3 publications

References 44 publications

Synthesis of L-methionine-loaded chitosan nanoparticles for controlled release and their in vitro and in vivo evaluation

Synthesis of L-methionine-loaded chitosan nanoparticles for controlled release and their in vitro and in vivo evaluation

Transcriptomic data analysis of melanocytes and melanoma cell lines of LAT transporter genes for precise medicine

Benefits of a prolonged-release amino acid mixture in four pregnant women with phenylketonuria

Contact Info

Product

Resources

About