Zobrazeno 1 - 10
of 26
pro vyhledávání: '"Scott D Bercury"'
Autor:
Mario A Cabrera-Salazar, Matthew Deriso, Scott D Bercury, Lingyun Li, John T Lydon, William Weber, Nilesh Pande, Mandy A Cromwell, Diane Copeland, John Leonard, Seng H Cheng, Ronald K Scheule
Publikováno v:
PLoS ONE, Vol 7, Iss 8, p e43310 (2012)
Neuropathic Gaucher disease (nGD), also known as type 2 or type 3 Gaucher disease, is caused by a deficiency of the enzyme glucocerebrosidase (GC). This deficiency impairs the degradation of glucosylceramide (GluCer) and glucosylsphingosine (GluSph),
Externí odkaz:
https://doaj.org/article/ec8f627f58cc40f6871e8639ff2b14b9
Autor:
Karen M Ashe, Dinesh Bangari, Lingyun Li, Mario A Cabrera-Salazar, Scott D Bercury, Jennifer B Nietupski, Christopher G F Cooper, Johannes M F G Aerts, Edward R Lee, Diane P Copeland, Seng H Cheng, Ronald K Scheule, John Marshall
Publikováno v:
PLoS ONE, Vol 6, Iss 6, p e21758 (2011)
The neuropathic glycosphingolipidoses are a subgroup of lysosomal storage disorders for which there are no effective therapies. A potential approach is substrate reduction therapy using inhibitors of glucosylceramide synthase (GCS) to decrease the sy
Externí odkaz:
https://doaj.org/article/cd3e8f6aa28047d9b5d2eaa2ee1c6e5d
Autor:
Christopher M. Treleaven, Robin J. Ziegler, Ernesto A. Salegio, Krystof S. Bankiewicz, John Bringas, Thomas J. Tamsett, Massimo S. Fiandaca, Scott D. Bercury, James Dodge, Piotr Hadaczek, Lamya S. Shihabuddin, Ronald K. Scheule
Publikováno v:
Experimental Neurology. 231:261-271
One treatment approach for lysosomal storage diseases (LSDs) is the systemic infusion of recombinant enzyme. Although this enzyme replacement is therapeutic for the viscera, many LSDs have central nervous system (CNS) components that are not adequate
Autor:
Scott D. Bercury, John Marshall, Nilesh Pande, Lisa Woodworth, Seng H. Cheng, Ronald K. Scheule, Jennifer B. Nietupski, Mark Bree, David W. Souza, Robin J. Ziegler, Michael Lukason, Elizabeth Meyers, Joseph W. Foley, Gregory D Hurlbut
Publikováno v:
Molecular Therapy. 18:1983-1994
Liver-directed gene therapy with adeno-associated virus (AAV) vectors effectively treats mouse models of lysosomal storage diseases (LSDs). We asked whether these results were likely to translate to patients. To understand to what extent preexisting
Autor:
Joseph W. Foley, Robin J. Ziegler, Scott D. Bercury, Patrick Finn, Seng H. Cheng, Ronald K. Scheule
Publikováno v:
Molecular Therapy. 18(9):1584-1591
Due to the lack of acid alpha-glucosidase (GAA) activity, Pompe mice develop glycogen storage pathology and progressive skeletal muscle dysfunction with age. Applying either gene or enzyme therapy to reconstitute GAA levels in older, symptomatic Pomp
Autor:
Gabriele S. V. Campanella, Andrew M. Tager, Peter LaCamera, Richard L. Kradin, Timothy S. Blackwell, Andrew D. Luster, Carol P. Leary, Vasiliy V. Polosukhin, Long-Hai Zhao, Hideo Sakamoto, Scott D. Bercury
Publikováno v:
American Journal of Respiratory Cell and Molecular Biology. 31:395-404
Pulmonary fibrosis is an enigmatic and devastating disease with few treatment options, now thought to result from abnormal wound healing in the lung in response to injury. We have previously noted a role for the chemokine interferon gamma-inducible p
Publikováno v:
Plant Molecular Biology. 47:341-351
Doppia (Dop) transposable elements were first identified from element termini found in the upstream portions of certain alleles of the pl1 and r1 loci of maize. At the r1 locus, these Dop end sequences are present in a region called σ, which functio
Autor:
Jennifer H. Dufour, Andrew M. Tager, Katayoon Goodarzi, Scott D. Bercury, Ulrich H. von Andrian, Andrew D. Luster
Publikováno v:
The Journal of Experimental Medicine
Leukotriene B4 (LTB4) is a potent chemoattractant active on multiple leukocytes, including neutrophils, macrophages, and eosinophils, and is implicated in the pathogenesis of a variety of inflammatory processes. A seven transmembrane–spanning, G pr
Autor:
John P. Leonard, Scott D. Bercury, Matthew DeRiso, Ronald K. Scheule, Mandy Cromwell, Diane Copeland, Seng H. Cheng, Mario A. Cabrera-Salazar, John Lydon, Nilesh Pande, Lingyun Li, William Weber
Publikováno v:
PLoS ONE, Vol 7, Iss 8, p e43310 (2012)
PLoS ONE
PLoS ONE
Neuropathic Gaucher disease (nGD), also known as type 2 or type 3 Gaucher disease, is caused by a deficiency of the enzyme glucocerebrosidase (GC). This deficiency impairs the degradation of glucosylceramide (GluCer) and glucosylsphingosine (GluSph),
Autor:
Mario A. Cabrera-Salazar, Edward R. Lee, John Marshall, Karen M. Ashe, Ronald K. Scheule, Seng H. Cheng, Dinesh S. Bangari, Scott D. Bercury, Jennifer B. Nietupski, Christopher G.F. Cooper, Johannes M. F. G. Aerts, Diane Copeland, Lingyun Li
Publikováno v:
PLoS ONE, 6(6). Public Library of Science
PLoS ONE, 6(6), e21758
PLoS ONE, Vol 6, Iss 6, p e21758 (2011)
PLoS ONE
PLoS ONE, 6(6), e21758
PLoS ONE, Vol 6, Iss 6, p e21758 (2011)
PLoS ONE
The neuropathic glycosphingolipidoses are a subgroup of lysosomal storage disorders for which there are no effective therapies. A potential approach is substrate reduction therapy using inhibitors of glucosylceramide synthase (GCS) to decrease the sy
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::129776ee0ffbc2da5303ab5c1fd1ac95
https://dare.uva.nl/personal/pure/en/publications/iminosugarbased-inhibitors-of-glucosylceramide-synthase-increase-brain-glycosphingolipids-and-survival-in-a-mouse-model-of-sandhoff-disease(428c7147-b4f9-44ef-b737-43bc29216353).html
https://dare.uva.nl/personal/pure/en/publications/iminosugarbased-inhibitors-of-glucosylceramide-synthase-increase-brain-glycosphingolipids-and-survival-in-a-mouse-model-of-sandhoff-disease(428c7147-b4f9-44ef-b737-43bc29216353).html