Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2000 Dec;23(8):805-18.
doi: 10.1023/a:1026708618507.

Branched-chain L-amino acid metabolism in classical maple syrup urine disease after orthotopic liver transplantation

A Bodner-Leidecker  1 , U Wendel, J M Saudubray, P Schadewaldt
Affiliations
Case Reports

Branched-chain L-amino acid metabolism in classical maple syrup urine disease after orthotopic liver transplantation

A Bodner-Leidecker et al. J Inherit Metab Dis. 2000 Dec.

Abstract

We characterized the effect of orthotopic liver transplantation on the catabolism of branched-chain L-amino acids in a female patient with classical form of maple syrup urine disease. Transplantation was performed at the age of 7.4 years due to a terminal liver failure triggered by a hepatitis A infection. Since then, the patient is on an unrestricted diet and plasma concentrations of branched-chain L-amino and 2-oxo acids are stable, yet at moderately increased levels (2- to 3-fold of control). L-Alloisoleucine concentrations, however, remained remarkably elevated (> 5-fold of control). In vivo catabolism was investigated by measuring the metabolic L-alloisoleucine clearance and whole-body leucine oxidation in the postabsorptive state. In an oral loading test with 580 micromol alloisoleucine per kg body wt, the L-alloisoleucine elimination rate constant (0.067 h(-1)) was in the normal range (0.069+/-0.012 h(-1), n = 4). In an oral L-[1-13C]leucine load (38 micromol/kg body wt), 19.5% of the tracer dose applied was recovered in exhaled 13CO2 versus 18.9+/-3.6% in healthy subjects (n = 10). Thus, the patient exhibited obviously normal whole-body catabolic rates although branched-chain L-amino acid oxidation was confined to the liver transplant. Most likely, the enhanced substrate supply from extrahepatic sources led to an elevation of the plasma concentrations and thus induced a compensatory enhancement of the metabolic flux through the branched-chain 2-oxo acid dehydrogenase complex in the intact liver tissue.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Pediatr Res. 1991 Nov;30(5):430-4 - PubMed
    1. Eur J Appl Physiol Occup Physiol. 1989;59(3):159-67 - PubMed
    1. Am J Physiol. 1997 Feb;272(2 Pt 1):E233-8 - PubMed
    1. Lab Res Methods Biol Med. 1984;9:1-287 - PubMed
    1. J Appl Physiol (1985). 1998 Nov;85(5):1744-52 - PubMed

Publication types