Targeted Metabolomic Analysis of a Mucopolysaccharidosis IIIB Mouse Model Reveals an Imbalance of Branched-Chain Amino Acid and Fatty Acid Metabolism

Targeted Metabolomic Analysis of a Mucopolysaccharidosis IIIB Mouse Model Reveals an Imbalance of Branched-Chain Amino Acid and Fatty Acid Metabolism

Mucopolysaccharidoses (MPSs) are inherited disorders of the glycosaminoglycan (GAG) metabolism. The defective digestion of GAGs within the intralysosomal compartment of affected patients leads to a broad spectrum of clinical manifestations ranging from cardiovascular disease to neurological impairme...

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Bibliographic Details
Main Authors: Valeria De Pasquale, Marianna Caterino, Michele Costanzo, Roberta Fedele, Margherita Ruoppolo, Luigi Michele Pavone
Format: Article
Language:English
Published: MDPI AG 2020-06-01
Series:International Journal of Molecular Sciences
Subjects:
lysosomal storage diseases
mucopolysaccharidosis IIIB
metabolomics
branched-chain amino acids
acylcarnitines
fatty acids
Online Access:https://www.mdpi.com/1422-0067/21/12/4211

Internet

https://www.mdpi.com/1422-0067/21/12/4211

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