Lipid changes in the metabolome of a single case study with maple syrup urine disease (MSUD) after five days of improved diet adherence of controlled branched-chain amino acids (BCAA)

Lipid changes in the metabolome of a single case study with maple syrup urine disease (MSUD) after five days of improved diet adherence of controlled branched-chain amino acids (BCAA)

Background: Distinguishing systemic metabolic disruptions in maple syrup urine disease (MSUD) beyond amino acid pathways is under-investigated, yet important to understanding disease pathology and treatment options. Methods: An adolescent female (15 years) with MSUD without liver transplant, attende...

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Bibliographic Details
Main Authors: Teresa D. Douglas, L. Kristin Newby, Julie Eckstrand, Douglas Wixted, Rani H. Singh
Format: Article
Language:English
Published: Elsevier 2020-12-01
Series:Molecular Genetics and Metabolism Reports
Subjects:
MSUD
Maple syrup urine disease
Branched-chain amino acids
Lipids
Metabolomics
Peroxisomes
Online Access:http://www.sciencedirect.com/science/article/pii/S2214426920300975

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http://www.sciencedirect.com/science/article/pii/S2214426920300975

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