苯丙酮尿症成人对蛋白质的需求以及甘氨肽与基于 l-氨基酸的产品的生物利用率比较。

IF 4.2 2区 医学 Q1 ENDOCRINOLOGY & METABOLISM Journal of Inherited Metabolic Disease Pub Date : 2024-10-15 DOI:10.1002/jimd.12806
Abrar Turki, Sylvia Stockler-Ipsiroglu, Sandra Sirrs, Jennifer Branov, Taryn Bosdet, Rajavel Elango
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引用次数: 0

摘要

背景:苯丙酮尿症(PKU)是由苯丙氨酸羟化酶缺乏引起的。治疗方法主要是低苯丙氨酸饮食和以 l-AA 为基础的氨基酸产品(l-AA)。尚未直接确定患有 PKU 的成人对蛋白质的需求量。目前有一种含有糖化麦角肽(GMP)和低苯丙氨酸的配方,但尚未经过蛋白质合成优化测试:目的:使用指示性氨基酸氧化(IAAO)技术确定患有 PKU 的成人对蛋白质的需求量以及 GMP-AA 在这些患者中的生物利用率:在实验1中,每位参与者被分配到7个不同的l-AA摄入量(范围:0.1-1.8克/公斤/天)。在实验 2 中,同样的患者参与了 4 次 GMP-AA 摄入(范围:0.1-0.9 克/千克/天)。以 l-[1-13C]-lysine 作为指示氨基酸并将其氧化为 13CO2 的 IAAO 方法被用作蛋白质合成的主要指标。通过断点确定蛋白质需求量,并通过比较 GMP-AA 与 l-AA 的 13CO2 斜率确定生物利用率:六名患有 PKU 的成年人(4 男 2 女)在两次实验中共完成了 54 个研究日。蛋白质的估计平均需要量(EAR)被确定为 1.11 克/千克/天(R2 = 0.20)。GMP-AA 蛋白质的生物利用率被测定为 99.98%,与 l-AA 相比很高,接近 100%;但这一结果仅适用于测试的 GMP-AA 混合物:据我们所知,这是第一项直接确定蛋白质定量需求的研究,表明目前针对患有 PKU 的成年人的蛋白质建议可能被低估了。GMP-AA混合物中蛋白质的生物利用率很高,是PKU成人蛋白质合成的最佳选择。
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Protein requirements in adults with phenylketonuria and bioavailability of glycomacropeptide compared to an l-amino acid-based product.

Background: Phenylketonuria (PKU) is caused by phenylalanine hydroxylase deficiency. Treatment is primarily a low-Phe diet combined with l-amino acid-based products (l-AA). Protein requirements in adults with PKU have not been directly determined. A formula with glycomacropeptide (GMP) and low phenylalanine is available, yet untested for optimal protein synthesis.

Objectives: To determine the protein requirements in adults with PKU and the bioavailability of GMP-AA in the same patients using the indicator amino acid oxidation (IAAO) technique.

Methods: Each participant was allocated to 7 separate l-AA intakes (range: 0.1-1.8 g/kg/day) in Experiment 1. In Experiment 2, the same patients participated in 4 GMP-AA intakes (range: 0.1-0.9 g/kg/day). The IAAO method with l-[1-13C]-lysine as the indicator amino acid and its oxidation to 13CO2 was used as the primary indicator of protein synthesis. Protein requirements were identified with a breakpoint, and bioavailability was determined by comparing 13CO2 slope from GMP-AA versus l-AA.

Results: Six adults with PKU (4 M: 2F) completed a total of 54 study days over the 2 experiments. The estimated average requirement (EAR) for protein was determined to be 1.11 g/kg/day (R2 = 0.20). The bioavailability of protein from GMP-AA was determined to be 99.98%, which was high and near to 100% comparable to l-AA; although, the results apply only to the tested GMP-AA blend.

Conclusions: To our knowledge, this is the first study to directly define a quantitative protein requirement and indicates that current PKU protein recommendations for adults with PKU may be underestimated. The bioavailability of protein in the GMP-AA blend was high and optimal for protein synthesis in adults with PKU.

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来源期刊
Journal of Inherited Metabolic Disease
Journal of Inherited Metabolic Disease 医学-内分泌学与代谢
CiteScore
9.50
自引率
7.10%
发文量
117
审稿时长
4-8 weeks
期刊介绍: The Journal of Inherited Metabolic Disease (JIMD) is the official journal of the Society for the Study of Inborn Errors of Metabolism (SSIEM). By enhancing communication between workers in the field throughout the world, the JIMD aims to improve the management and understanding of inherited metabolic disorders. It publishes results of original research and new or important observations pertaining to any aspect of inherited metabolic disease in humans and higher animals. This includes clinical (medical, dental and veterinary), biochemical, genetic (including cytogenetic, molecular and population genetic), experimental (including cell biological), methodological, theoretical, epidemiological, ethical and counselling aspects. The JIMD also reviews important new developments or controversial issues relating to metabolic disorders and publishes reviews and short reports arising from the Society''s annual symposia. A distinction is made between peer-reviewed scientific material that is selected because of its significance for other professionals in the field and non-peer- reviewed material that aims to be important, controversial, interesting or entertaining (“Extras”).
期刊最新文献
Issue Information Potential therapeutic uses of L-citrulline beyond genetic urea cycle disorders Epidemiology and economic burden of Wilson disease in France: A nationwide population-based study. Human glyoxylate metabolism revisited: New insights pointing to multi-organ involvement with implications for siRNA-based therapies in primary hyperoxaluria. My path to citrin deficiency.
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