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Journal article
Stable isotopes and LC-MS for monitoring metabolic disturbances in Friedreich's ataxia platelets
Bioanalysis, v 7(15), pp 1843-1855
2015
PMID: 26295986
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Friedreich's ataxia (FRDA) is an autosomal recessive disease with metabolic abnormalities that have been proposed to play an important role in the resulting neurodegeneration and cardiomyopathy. The inability to access the highly affected neuronal and cardiac tissues has hampered metabolic evaluation and biomarker development.
Employment of a LC-MS-based method to determine whether platelets isolated from patients with FRDA exhibit differentiable metabolism compared with healthy controls.
Isotopologue analysis showed a marked decrease in glucose incorporation with a concomitant increase in palmitate-derived acyl-CoA thioesters in FRDA platelets compared with controls.
Our findings demonstrate that platelets can be used as a surrogate tissue for in vivo biomarker studies to monitor new therapeutic approaches for the treatment of FRDA.
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Details
- Title
- Stable isotopes and LC-MS for monitoring metabolic disturbances in Friedreich's ataxia platelets
- Creators
- Andrew J Worth - University of PennsylvaniaSankha S Basu - Translational TherapeuticsEric C Deutsch - Translational TherapeuticsWei-Ting Hwang - University of PennsylvaniaNathaniel W Snyder - Drexel UniversityDavid R Lynch - Departments of Neurology & Pediatrics, The Children's Hospital of Philadelphia & the Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USAIan A Blair - University of Pennsylvania
- Publication Details
- Bioanalysis, v 7(15), pp 1843-1855
- Grant note
- P30ES013508 / NIEHS NIH HHS T32 GM008076 / NIGMS NIH HHS P30 ES013508 / NIEHS NIH HHS T32 ES019851 / NIEHS NIH HHS R21NS087343 / NINDS NIH HHS R21 NS087343 / NINDS NIH HHS T32ES019851 / NIEHS NIH HHS
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- A.J. Drexel Autism Institute
- Web of Science ID
- WOS:000360296400004
- Scopus ID
- 2-s2.0-84940206935
- Other Identifier
- 991019167866804721
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- Collaboration types
- Domestic collaboration
- Web of Science research areas
- Biochemical Research Methods
- Chemistry, Analytical