Zobrazeno 1 - 10
of 79
pro vyhledávání: '"Evelyn Ralston"'
Publikováno v:
Molecular Therapy: Methods & Clinical Development, Vol 18, Iss , Pp 199-214 (2020)
Pompe disease, a deficiency of glycogen-degrading lysosomal acid alpha-glucosidase (GAA), is a disabling multisystemic illness that invariably affects skeletal muscle in all patients. The patients still carry a heavy burden of the disease, despite th
Externí odkaz:
https://doaj.org/article/665cf73afe1b482cb49770fa9bb20386
Autor:
Sarah Oddoux, Davide Randazzo, Aster Kenea, Bruno Alonso, Kristien J. M. Zaal, Evelyn Ralston
Publikováno v:
Frontiers in Cell and Developmental Biology, Vol 7 (2019)
Differentiated mammalian cells and tissues, such as skeletal muscle fibers, acquire an organization of Golgi complex and microtubules profoundly different from that in proliferating cells and still poorly understood. In adult rodent skeletal muscle,
Externí odkaz:
https://doaj.org/article/4c5562be91434045873f3fd82bbad05a
Autor:
Francoise Fouquet, Jia Min Zhou, Evelyn Ralston, Kerren Murray, Frédéric Troalen, Ella Magal, Olivier Robain, M. Dubois-Dalcq, Patrick Aubourg
Publikováno v:
Neurobiology of Disease, Vol 3, Iss 4, Pp 271-285 (1997)
The gene mutated in X-linked adrenoleukodystrophy (ALD), a progressive demyelinating disease, codes for a protein (ALDP) involved in very-long-chain fatty acid (VLCFA) transport. The expression of ALDP and of two peroxisomal enzymes involved in β-ox
Externí odkaz:
https://doaj.org/article/86226d97e6cb4bc894b6577f32e9a5fe
Autor:
Kristien J M Zaal, Ericka Reid, Kambiz Mousavi, Tan Zhang, Amisha Mehta, Elisabeth Bugnard, Vittorio Sartorelli, Evelyn Ralston
Publikováno v:
PLoS ONE, Vol 6, Iss 12, p e29057 (2011)
A wave of structural reorganization involving centrosomes, microtubules, Golgi complex and ER exit sites takes place early during skeletal muscle differentiation and completely remodels the secretory pathway. The mechanism of these changes and their
Externí odkaz:
https://doaj.org/article/7db8bde66d874ac5a42f5c01bfc7f9f2
Autor:
Shoichi Takikita, Cynthia Schreiner, Rebecca Baum, Tao Xie, Evelyn Ralston, Paul H Plotz, Nina Raben
Publikováno v:
PLoS ONE, Vol 5, Iss 12, p e15239 (2010)
PGC-1α is a transcriptional co-activator that plays a central role in the regulation of energy metabolism. Our interest in this protein was driven by its ability to promote muscle remodeling. Conversion from fast glycolytic to slow oxidative fibers
Externí odkaz:
https://doaj.org/article/29f1fd6a68f84f48a5f2b0ac27a9d057
Publikováno v:
Molecular Therapy: Methods & Clinical Development, Vol 18, Iss, Pp 199-214 (2020)
Molecular Therapy. Methods & Clinical Development
Molecular Therapy. Methods & Clinical Development
Pompe disease, a deficiency of glycogen-degrading lysosomal acid alpha-glucosidase (GAA), is a disabling multisystemic illness that invariably affects skeletal muscle in all patients. The patients still carry a heavy burden of the disease, despite th
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::535c96e9e7770a64909cbaba2a0f2c29
http://www.sciencedirect.com/science/article/pii/S2329050120301133
http://www.sciencedirect.com/science/article/pii/S2329050120301133
The mdx mouse (C57BL/10ScSn-DMDmdx/J) is the oldest model of Duchenne muscular dystrophy (DMD). Mdx remains popular and has not been replaced by newer mouse models, despite criticisms that mdx has a nearly normal lifespan and mild pathology while DMD
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::b994556ecd3a4e0380fae7aaa83ee29b
https://doi.org/10.1101/2021.07.16.452553
https://doi.org/10.1101/2021.07.16.452553
Autor:
Peter Schjerling, Thomas E. Jensen, Jonas R. Knudsen, Evelyn Ralston, Agnete B. Madsen, Lykke Sylow, Carlos Henríquez-Olguín, Yeliz Angin, Kristien J. M. Zaal
Publikováno v:
American Journal of Physiology-Endocrinology and Metabolism. 315:E110-E125
Studies in skeletal muscle cell cultures suggest that the cortical actin cytoskeleton is a major requirement for insulin-stimulated glucose transport, implicating the β-actin isoform, which in many cell types is the main actin isoform. However, it i
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a0b90392268cb0a605c93e93bfe977ed
https://doi.org/10.1152/ajpendo.00392.2017
https://doi.org/10.1152/ajpendo.00392.2017
Autor:
Evelyn Ralston, Davide Randazzo, Aster Kenea, Bruno Alonso, Kristien J. M. Zaal, Sarah Oddoux
Publikováno v:
Frontiers in Cell and Developmental Biology
Frontiers in Cell and Developmental Biology, Vol 7 (2019)
Frontiers in Cell and Developmental Biology, Vol 7 (2019)
Differentiated mammalian cells and tissues, such as skeletal muscle fibers, acquire an organization of Golgi complex and microtubules profoundly different from that in proliferating cells and still poorly understood. In adult rodent skeletal muscle,
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6712c77bd96caedda20a297d1b8a4206
https://doi.org/10.3389/fcell.2019.00176
https://doi.org/10.3389/fcell.2019.00176
Autor:
Yi Lun, Anju Nair, Anadina Garcia, Jessie Feng, Kenneth J. Valenzano, Tuske Steven, Rosa Puertollano, Richie Khanna, Michelle Frascella, Su Xu, Russell Gotschall, Jose A. Martina, Maria Cecilia Della Valle, Evelyn Ralston, Adriane Schilling, Hung V. Do, Nina Raben, Abdul S. Ponery, Rebecca Soska
Publikováno v:
JCI Insight. 4
Pompe disease is a rare inherited disorder of lysosomal glycogen metabolism due to acid α-glucosidase (GAA) deficiency. Enzyme replacement therapy (ERT) using alglucosidase alfa, a recombinant human GAA (rhGAA), is the only approved treatment for Po
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2f12ff3199f2c555ef7abb754419f457
https://doi.org/10.1172/jci.insight.125358
https://doi.org/10.1172/jci.insight.125358