Phenylbutyrate improves nitrogen disposal via an alternative pathway without eliciting an increase in protein breakdown and catabolism in control and ornithine transcarbamylase–deficient patients  

Oleh:

Marini, Juan C. ; Lanpher, Brendan C ; Scaglia, Fernando ; O'Brien, William E. ; Sun, Qin

Jenis: Article from Journal - ilmiah internasional
Dalam koleksi: The American Journal of Clinical Nutrition vol. 93 no. 06 (Jun. 2011), page 1248-1254 .
Topik: PROTEIN; Urea Cycle Disorder; Prolonged Phenylbutyrate
Fulltext: Am J Clin Nutr-2011-Marini-1248-54.pdf (126.19KB)

Ketersediaan

Perpustakaan FK Nomor Panggil: A07.K.2011.01 Non-tandon: 1 (dapat dipinjam: 0) Tandon: tidak ada

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Isi artikel

Background: Phenylbutyrate is a drug used in patients with urea cycle disorder to elicit alternative pathways for nitrogen disposal. However, phenylbutyrate administration decreases plasma branched-chain amino acid (BCAA) concentrations, and previous research suggests that phenylbutyrate administration may increase leucine oxidation, which would indicate increased protein degradation and net protein loss. Objective: We investigated the effects of phenylbutyrate administration on whole-body protein metabolism, glutamine, leucine, and urea kinetics in healthy and ornithine transcarbamylase–deficient (OTCD) subjects and the possible benefits of BCAA supplementation during phenylbutyrate therapy. Design: Seven healthy control and 7 partial-OTCD subjects received either phenylbutyrate or no treatment in a crossover design. In addition, the partial-OTCD and 3 null-OTCD subjects received phenylbutyrate and phenylbutyrate plus BCAA supplementation. A multitracer protocol was used to determine the whole-body fluxes of urea and amino acids of interest. Results: Phenylbutyrate administration reduced ureagenesis by ˜15% without affecting the fluxes of leucine, tyrosine, phenylalanine, or glutamine and the oxidation of leucine or phenylalanine. The transfer of 15N from glutamine to urea was reduced by 35%. However, a reduction in plasma concentrations of BCAAs due to phenylbutyrate treatment was observed. BCAA supplementation did not alter the respective baseline fluxes. Conclusions: Prolonged phenylbutyrate administration reduced ureagenesis and the transfer of 15N from glutamine to urea without parallel reductions in glutamine flux and concentration. There were no changes in total-body protein breakdown and amino acid catabolism, which suggests that phenylbutyrate can be used to dispose of nitrogen effectively without adverse effects on body protein economy.

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