Cell cycle disturbances in mucopolysaccharidoses: Transcriptomic and experimental studies on cellular models.

ABSTRACT: Mucopolysaccharidoses (MPS) are a group of lysosomal storage diseases caused by defects in genes coding for proteins involved in degradation of glycosaminoglycans (GAGs). These complex carbohydrates accumulate in cells causing their serious dysfunctions. Apart from the physical GAG storage, secondary and tertiary changes may contribute significantly to the pathomechanism of the disease. Among processes which were not systematically investigated in MPS cells to date there is the cell cycle. Here, we studied perturbances in this crucial cellular process in majority of MPS types. Transcriptomic analyses indicated that expression of many genes coding for proteins involved in the cell cycle is dysregulated in all tested MPS cells. Importantly, levels of transcripts of particular genes were chan

SUBMITTER: Brokowska J 

PROVIDER: S-EPMC9597211 | biostudies-literature | 2022 Sep

REPOSITORIES: biostudies-literature