Preclinical Research in Glycogen Storage Diseases: A Comprehensive Review of Current Animal Models.
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ABSTRACT:
SUBMITTER: Almodovar-Paya A
PROVIDER: S-EPMC7766110 | biostudies-literature | 2020 Dec
REPOSITORIES: biostudies-literature
Publications
Preclinical Research in Glycogen Storage Diseases: A Comprehensive Review of Current Animal Models.
Almodóvar-Payá Aitana A Villarreal-Salazar Mónica M de Luna Noemí N Nogales-Gadea Gisela G Real-Martínez Alberto A Andreu Antoni L AL Martín Miguel Angel MA Arenas Joaquin J Lucia Alejandro A Vissing John J Krag Thomas T Pinós Tomàs T
International journal of molecular sciences 20201217 24
GSD are a group of disorders characterized by a defect in gene expression of specific enzymes involved in glycogen breakdown or synthesis, commonly resulting in the accumulation of glycogen in various tissues (primarily the liver and skeletal muscle). Several different GSD animal models have been found to naturally present spontaneous mutations and others have been developed and characterized in order to further understand the physiopathology of these diseases and as a useful tool to evaluate po ...[more]
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