alexa Abstract | Glycogen Exocytosis from Cultured Pompe Skin Fibroblasts

Translational Biomedicine
Open Access

OMICS International organises 3000+ Global Conferenceseries Events every year across USA, Europe & Asia with support from 1000 more scientific Societies and Publishes 700+ Open Access Journals which contains over 50000 eminent personalities, reputed scientists as editorial board members.

Open Access Journals gaining more Readers and Citations

700 Journals and 15,000,000 Readers Each Journal is getting 25,000+ Readers

This Readership is 10 times more when compared to other Subscription Journals (Source: Google Analytics)

Research Article Open Access


Objective: Pompe disease is a progressive form of muscular dystrophy caused by a deficiency in the lysosomal enzyme α-glucosidase (GAA), and leads to the accumulation of glycogen in affected cells. Enzyme replacement therapy is approved to treat infantile-onset Pompe disease, but this is not completely effective, necessitating the development of new therapeutic strategies. Exocytosis involves the fusion of intracellular vesicles with the cell surface and the release of vesicular content, and is a mechanism that could be used in Pompe disease to remove stored glycogen from affected cells. The exocytosis of storage material from Pompe patient cells into circulation could result in glycogen degradation by other amylases (i.e. not GAA) and this could be developed in the future as a new or adjunct therapeutic strategy.

Methods: A sensitive mass spectrometry assay was used to quantify glycogen in cell extracts and the culture media from confluent Pompe skin fibroblasts.

Results: Four percent of vesicular glycogen was exocytosed after 2 hours in culture. This natural process of glycogen exocytosis was enhanced in sub-confluent Pompe cells, which released >80% of glycogen after 2 hours in culture.

Conclusion: Under appropriate conditions exocytosis can release most of the stored glycogen in Pompe skin fibroblasts, identifying a potential target for therapeutic intervention.

To read the full article Peer-reviewed Article PDF image | Peer-reviewed Full Article image

Author(s): Christopher T TurnerMaria Fuller John J Hopwood Peter J Meikle Doug A Brooks


Pompe, Lysosomal storage disorder, Glycogen, Exocytosis, Substrate degradation, Autoimmune Skin Diseases,Translational Research and Clinical Intervention

Peer Reviewed Journals
Make the best use of Scientific Research and information from our 700 + peer reviewed, Open Access Journals
International Conferences 2017-18
Meet Inspiring Speakers and Experts at our 3000+ Global Annual Meetings

Contact Us

© 2008-2017 OMICS International - Open Access Publisher. Best viewed in Mozilla Firefox | Google Chrome | Above IE 7.0 version