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Citations to this article

Circumvention of defective neutral amino acid transport in Hartnup disease using tryptophan ethyl ester.
A J Jonas, I J Butler
A J Jonas, I J Butler
Published July 1, 1989
Citation Information: J Clin Invest. 1989;84(1):200-204. https://doi.org/10.1172/JCI114141.
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Research Article Article has an altmetric score of 3

Circumvention of defective neutral amino acid transport in Hartnup disease using tryptophan ethyl ester.

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Abstract

Tryptophan ethyl ester, a lipid-soluble tryptophan derivative, was used to bypass defective gastrointestinal neutral amino acid transport in a child with Hartnup disease. The child's baseline tryptophan concentrations in serum (20 +/- 6 microM) and cerebrospinal fluid (1.0 +/- 0.2 microM) were persistently less than 50% of normal values. Cerebrospinal fluid 5-hydroxyindoleacetic acid (5-HIAA), a serotonin metabolite, was also less than 50% of normal (21 +/- 2 ng/ml). Serum tryptophan concentrations increased only modestly and briefly after an oral challenge with 200 mg/kg of oral L-tryptophan, reflecting the absorptive defect. An oral challenge with 200 mg/kg of tryptophan ethyl ester resulted in a prompt increase in serum tryptophan to a peak of 555 microM. Sustained treatment with 20 mg/kg q6h resulted in normalization of serum (66 +/- 15 microM) and cerebrospinal fluid tryptophan concentrations (mean = 2.3 microM). Cerebrospinal fluid 5-HIAA increased to more normal concentrations (mean = 33 ng/ml). No toxicity was observed over an 8-mo period of treatment, chronic diarrhea resolved, and body weight, which had remained unchanged for 7 mo before ester therapy, increased by approximately 26%. We concluded that tryptophan ethyl ester is effective at circumventing defective gastrointestinal neutral amino acid transport and may be useful in the treatment of Hartnup disease.

Authors

A J Jonas, I J Butler

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Total citations by year

Year: 2025 2023 2021 2020 2016 2015 2014 2013 2012 2011 2010 2009 2008 2006 2005 2001 1998 Total
Citations: 1 1 1 1 1 4 1 1 1 3 24 1 2 2 2 1 1 48
Citation information
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Citations to this article in year 2010 (24)

Title and authors Publication Year
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2010
Microorganisms Responsible for Neonatal Diarrhea
O'Ryan ML, Nataro JP, Cleary TG
Infectious Diseases of the Fetus and Newborn 2010

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