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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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Bibliografski detalji
Izdano u:Int J Mol Sci
Glavni autori: De Pasquale, Valeria, Caterino, Marianna, Costanzo, Michele, Fedele, Roberta, Ruoppolo, Margherita, Pavone, Luigi Michele
Format: Artigo
Jezik:Inglês
Izdano: MDPI 2020
Teme:
Online pristup: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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