ArticleJIMD reports2019
Large neutral amino acid status in association with P:T ratio and diet in adult and pediatric patients with phenylketonuria.
Article in JIMD reports, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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3 citing papers in PubMed, 6 citations in OpenAlex.
- Measurement of Protein Synthesis Rate in Rat by [Molecular imaging and biology · 2023Article
- Physicochemical Characterization and In Vitro Digestibility Study of an In Silico Designed Recombinant Protein Enriched with Large Neutral Amino Acids and Lacking Phenylalanine for Phenylketonuria.The protein journal · 2022Article
- Large neutral amino acid status in association with P:T ratio and diet in adult and pediatric patients with phenylketonuria.JIMD reports · 2019Article
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundIntake of large neutral amino acids (LNAA) may inhibit phenylalanine (PHE) transport across the blood brain barrier and assist with blood PHE control in patients with phenylketonuria (PKU). We evaluated the interrelationship between LNAA in plasma and diet on Phe:Tyr (P:T) ratio in patients with PKU and the influence of dietary factors on plasma LNAA markers.
methodsPlasma amino acid values and 3-day food record analysis from two studies (34 male/30 female; age 4.6-47 years) were examined. For pediatrics (<18 years) and adults (≥18 years) the relationship between P:T ratio, plasma LNAA, and dietary intake patterns were investigated.
resultsDietary factors influencing P:T ratio included intake of total protein (g/kg), medical food (MF) protein (g/kg, % below Rx), and LNAA (g) in the full cohort (
conclusionP:T ratio is significantly tied to dietary LNAA, adherence to MF Rx, and plasma LNAA concentrations. Additionally, P:T ratio and valine may be effective clinical proxies for determining LNAA metabolic balance and LNAA quality of the diet in patients with PKU.
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