The imbalance of brain large-chain aminoacid availability in amyotrophic lateral sclerosis patients treated with high doses of branched-chain aminoacids

A. Bastone, A. Micheli, E. Beghi, M. Salmona

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Abstract

Following the report of an increased mortality among patients with amyotrophic lateral sclerosis given high daily doses of branched-chain aminoacids, we assessed the plasma concentrations of large neutral aminoacids and glutamic acid and the large neutral aminoacid brain influx in 24 amyotrophic lateral sclerosis patients receiving placebo or branched-chain aminoacids (l-leucine 12 g, l-isoleucine 6 g, l-valine 6 g daily), in 15 untreated amyotrophic lateral sclerosis patients and in 15 healthy volunteers. The branched-chain aminoacid plasma concentrations increased three- to six-fold in the treated group compared to the patients receiving placebo or no treatment and to the healthy controls. Plasma glutamic acid concentrations in healthy volunteers were 51.59±7.53 nmol/ml while in the amyotrophic lateral sclerosis patients receiving no treatment, placebo or branched-chain aminoacids were 92.33±12.15 nmol/ml, 91.21±15.86 nmol/ml and 95.08±17.96 nmol/ml respectively. The glutamic acid concentration was significantly higher (P

Original languageEnglish
Pages (from-to)467-472
Number of pages6
JournalNeurochemistry International
Volume27
Issue number6
DOIs
Publication statusPublished - 1995

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Amyotrophic Lateral Sclerosis
Brain
Glutamic Acid
Placebos
Healthy Volunteers
Isoleucine
Valine
Leucine
Mortality
Therapeutics

ASJC Scopus subject areas

  • Cell Biology
  • Cellular and Molecular Neuroscience
  • Molecular Biology

Cite this

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abstract = "Following the report of an increased mortality among patients with amyotrophic lateral sclerosis given high daily doses of branched-chain aminoacids, we assessed the plasma concentrations of large neutral aminoacids and glutamic acid and the large neutral aminoacid brain influx in 24 amyotrophic lateral sclerosis patients receiving placebo or branched-chain aminoacids (l-leucine 12 g, l-isoleucine 6 g, l-valine 6 g daily), in 15 untreated amyotrophic lateral sclerosis patients and in 15 healthy volunteers. The branched-chain aminoacid plasma concentrations increased three- to six-fold in the treated group compared to the patients receiving placebo or no treatment and to the healthy controls. Plasma glutamic acid concentrations in healthy volunteers were 51.59±7.53 nmol/ml while in the amyotrophic lateral sclerosis patients receiving no treatment, placebo or branched-chain aminoacids were 92.33±12.15 nmol/ml, 91.21±15.86 nmol/ml and 95.08±17.96 nmol/ml respectively. The glutamic acid concentration was significantly higher (P",
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AU - Micheli, A.

AU - Beghi, E.

AU - Salmona, M.

PY - 1995

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