Validation of Plasma Branched Chain Amino Acids as Biomarkers in Huntington Disease
Authors:
- Fanny MochelFMFanny Mochel
- Sandra BenaichSBSandra Benaich
- Daniel RabierDRDaniel Rabier
- Alexandra DurrADAlexandra Durr
Open Access
- 1 February 2011
- journal article
- research article
- Published by American Medical Association (AMA) in Archives of Neurology
- Vol. 68 (2) , 264-271
Abstract
While investigating body weight in a cohort of 32 patients at an early stage of Huntington disease (HD) and presymptomatic HD gene carriers, we found a significant decrease in the plasma branched-chain amino acids (BCAA) valine, leucine, and isoleucine in the HD group compared with 21 healthy controls. This systemic metabolic defect, which is indicative of hypercatabolism, was associated with early body weight loss in the HD group.1 We wanted to (1) try to replicate our initial findings in a larger HD cohort, and (2) assess the feasibility of using plasma BCAA as a biomarker in HD, ie, in a less controlled research environment than the initial study.1Keywords
This publication has 3 references indexed in Scilit:
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- Early Energy Deficit in Huntington Disease: Identification of a Plasma Biomarker Traceable during Disease ProgressionPLOS ONE, 2007
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