Search

Your search keyword '"Moreland, Rodney"' showing total 4 results
Start Over Author "Moreland, Rodney" Journal molecular genetics & metabolism
4 results on '"Moreland, Rodney"'

3. Inhibition of glycogen biosynthesis via mTORC1 suppression as an adjunct therapy for Pompe disease

Author

Ashe, Karen M., Taylor, Kristin M., Chu, Qiuming, Meyers, Elizabeth, Ellis, Allen, Jingozyan, Varvara, Klinger, Katherine, Finn, Patrick F., Cooper, Christopher G.F., Chuang, Wei-Lien, Marshall, John, McPherson, John M., Mattaliano, Robert J., Cheng, Seng H., Scheule, Ronald K., and Moreland, Rodney J.

Subjects
*GLYCOGEN storage disease type II, *ETIOLOGY of diseases, *GLUCOSIDASES, *GLYCOGEN, *BIOSYNTHESIS, *RAPAMYCIN, *IMMUNOSUPPRESSIVE agents, *THERAPEUTICS
Abstract

Abstract: Pompe disease, also known as glycogen storage disease (GSD) type II, is caused by deficiency of lysosomal acid α-glucosidase (GAA). The resulting glycogen accumulation causes a spectrum of disease severity ranging from a rapidly progressive course that is typically fatal by 1–2years of age to a more slowly progressive course that causes significant morbidity and early mortality in children and adults. Recombinant human GAA (rhGAA) improves clinical outcomes with variable results. Adjunct therapy that increases the effectiveness of rhGAA may benefit some Pompe patients. Co-administration of the mTORC1 inhibitor rapamycin with rhGAA in a GAA knockout mouse reduced muscle glycogen content more than rhGAA or rapamycin alone. These results suggest mTORC1 inhibition may benefit GSDs that involve glycogen accumulation in muscle. [Copyright &y& Elsevier]

Published
2010
Full Text
View/download PDF

4. Antisense oligonucleotide-mediated suppression of muscle glycogen synthase 1 synthesis as an approach for substrate reduction therapy of Pompe disease.

Author

Clayton, Nicholas P., Nelson, Carol A., Weeden, Timothy, Taylor, Kristin M., Moreland, Rodney J., Scheule, Ronald K., Leger, Andrew J., Phillips, Lucy, Cheng, Seng H., and Wentworth, Bruce M.

Subjects
*GLYCOGEN storage disease type II, *ANTISENSE peptides, *OLIGONUCLEOTIDES, *IMMUNOSUPPRESSION, *GLYCOGEN synthase kinase, *THERAPEUTICS
Published
2015
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results