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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Vydáno v:Int J Mol Sci
Hlavní autoři: De Pasquale, Valeria, Caterino, Marianna, Costanzo, Michele, Fedele, Roberta, Ruoppolo, Margherita, Pavone, Luigi Michele
Médium: Artigo
Jazyk:Inglês
Vydáno: MDPI 2020
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On-line přístup: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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