top of page
Search

Glucocerebrosidase deficiency promotes protein aggregation through dysregulation of exosomes

We are proud to share with you another publication that has utilized Alpha Nano Tech services. Ruth E. Thomas and Evelyn S. Vincow from Department of Genome Sciences, University of Washington, Seattle, WA have shown in their PLOS Genetics paper a connection between Glucocerebrosidase deficiency and dysregulation of extracellular vesicles. Mutations in the glucosylceramidase beta (GBA) gene are strongly associated with neurodegenerative diseases marked by protein aggregation. A common explanation for the link between GBA mutations and protein aggregation is that lysosomal accumulation of glucosylceramide causes impaired autophagy. Proteomic analyses revealed that known autophagy substrates, which had severely impaired turnover in autophagy-deficient Atg7 mutants, showed little to no overall slowing of turnover or increase in abundance in Gba1b mutants. Likewise, Gba1b mutants did not have the marked impairment of mitochondrial protein turnover seen in mitophagy-deficient parkin mutants. Proteasome activity, microautophagy, and endocytic degradation also appeared unaffected in Gba1b mutants. However, the authors found striking changes in the turnover and abundance of proteins associated with extracellular vesicles (EVs), which have been proposed as vehicles for the spread of protein aggregates in neurodegenerative disease. These changes were specific to Gba1b mutants and did not represent an acceleration of normal aging. Their findings indicate that glucocerebrosidase deficiency causes pathogenic changes in EV metabolism and may promote the spread of protein aggregates through extracellular vesicles.


For more details read the full article HERE.

43 views0 comments

Recent Posts

See All

cGMP large scale manufacturing of EVs

We are proud and excited to announce that we are now offering large-scale cGMP exosome production and characterization services. Our state-of-the-art facility is equipped with the latest technology an

Comments


bottom of page