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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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Detalles Bibliográficos
Autores principales:	De Pasquale, Valeria, Caterino, Marianna, Costanzo, Michele, Fedele, Roberta, Ruoppolo, Margherita, Pavone, Luigi Michele
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2020
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7352355/ https://www.ncbi.nlm.nih.gov/pubmed/32545699 http://dx.doi.org/10.3390/ijms21124211

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