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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Veröffentlicht in:Int J Mol Sci
Hauptverfasser: De Pasquale, Valeria, Caterino, Marianna, Costanzo, Michele, Fedele, Roberta, Ruoppolo, Margherita, Pavone, Luigi Michele
Format: Artigo
Sprache:Inglês
Veröffentlicht: MDPI 2020
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Article
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC7352355/
https://ncbi.nlm.nih.gov/pubmed/32545699
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/ijms21124211
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