Zobrazeno 1 - 10
of 72
pro vyhledávání: '"Jin Song Shen"'
Autor:
Paulina Dabrowska‐Schlepp, Andreas Busch, Jin‐Song Shen, Rachel Y. Cheong, Lone Bruhn Madsen, Daniel Mascher, Raphael Schiffmann, Andreas Schaaf
Publikováno v:
JIMD Reports, Vol 64, Iss 6, Pp 460-467 (2023)
Abstract Fabry disease (FD, OMIM 301500) is a rare X‐linked inherited lysosomal storage disorder associated with reduced activities of α‐galactosidase A (aGal, EC 3.2.1.22). The current standard of care for FD is based on enzyme replacement ther
Externí odkaz:
https://doaj.org/article/022e2d6d730d4b30aa55b3a684353bb0
Autor:
William C. Hallows, Kristen Skvorak, Nick Agard, Nikki Kruse, Xiyun Zhang, Yu Zhu, Rachel C. Botham, Chinping Chng, Charu Shukla, Jessica Lao, Mathew Miller, Antoinette Sero, Judy Viduya, Moulay Hicham Alaoui Ismaili, Kerryn McCluskie, Raphael Schiffmann, Adam P. Silverman, Jin-Song Shen, Gjalt W. Huisman
Publikováno v:
Scientific Reports, Vol 13, Iss 1, Pp 1-16 (2023)
Abstract Fabry disease is caused by a deficiency of α-galactosidase A (GLA) leading to the lysosomal accumulation of globotriaosylceramide (Gb3) and other glycosphingolipids. Fabry patients experience significant damage to the heart, kidney, and blo
Externí odkaz:
https://doaj.org/article/b25ba72e3abd48129972a8895661aad7
Autor:
Jin-Song Shen, Uthra Balaji, Kunitoshi Shigeyasu, Yoshinaga Okugawa, Siamak Jabbarzadeh-Tabrizi, Taniqua S. Day, Erland Arning, John Marshall, Seng H. Cheng, Jinghua Gu, Raphael Schiffmann, Teodoro Bottiglieri, Ajay Goel
Publikováno v:
Molecular Genetics and Metabolism Reports, Vol 33, Iss , Pp 100919- (2022)
Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of α-galactosidase A and subsequent accumulation of glycosphingolipids with terminal α-D-galactosyl residues. The molecular process through which this abnormal metabolis
Externí odkaz:
https://doaj.org/article/1a8df2290a0e4d9c86cc9139ef242318
Autor:
Siamak Jabbarzadeh-Tabrizi, Michel Boutin, Taniqua S. Day, Mouna Taroua, Raphael Schiffmann, Christiane Auray-Blais, Jin-Song Shen
Publikováno v:
Journal of Lipid Research, Vol 61, Iss 11, Pp 1410-1423 (2020)
Fabry disease is caused by deficient activity of α-galactosidase A, an enzyme that hydrolyzes the terminal α-galactosyl moieties from glycolipids and glycoproteins, and subsequent accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb
Externí odkaz:
https://doaj.org/article/f6fe6c479ae44181953dc65f8c6f0431
Autor:
Weihua Tian, Zilu Ye, Shengjun Wang, Morten Alder Schulz, Julie Van Coillie, Lingbo Sun, Yen-Hsi Chen, Yoshiki Narimatsu, Lars Hansen, Claus Kristensen, Ulla Mandel, Eric Paul Bennett, Siamak Jabbarzadeh-Tabrizi, Raphael Schiffmann, Jin-Song Shen, Sergey Y. Vakhrushev, Henrik Clausen, Zhang Yang
Publikováno v:
Nature Communications, Vol 10, Iss 1, Pp 1-13 (2019)
Lysosomal replacement enzymes are taken up by cell surface receptors that recognize glycans, the effects of different glycan features are unknown. Here the authors present a gene engineering screen in CHO cells that allows custom N-glycan-decorated e
Externí odkaz:
https://doaj.org/article/61e178a4b78e40d9866c7f7e1c00ad4a
Publikováno v:
Petroleum Science, Vol 16, Iss 1, Pp 58-76 (2018)
Abstract We present a path morphology method to separate total rock pore space into matrix, fractures and vugs and derive their pore structure spectrum. Thus, we can achieve fine pore evaluation in fracture–vug reservoirs based on electric imaging
Externí odkaz:
https://doaj.org/article/a8c5948ec10b47b8b0f3a056c1cf7e71
Publikováno v:
Petroleum Science. 19:2027-2045
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::1a8772c0a9b5d96b5e9289836698c8ee
https://doi.org/10.1016/j.petsci.2022.08.005
https://doi.org/10.1016/j.petsci.2022.08.005
Publikováno v:
Applied Geophysics.
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::8c8ea2de36eca666a07d685dad09c9f1
https://doi.org/10.1007/s11770-022-0952-4
https://doi.org/10.1007/s11770-022-0952-4
Autor:
Anadina Garcia, Anthony Perry, Yi Lun, Lukas Martin, Leo B. Dungan, Jin-Song Shen, Yi Shuan Wu, Virginia D. Schmith, Franklin K. Johnson, Raphael Schiffmann, Richie Khanna, Rick Hamler, Anibh M. Das, Pai-Chi Tsai
Publikováno v:
Clinical Pharmacology in Drug Development
Approved therapies for Fabry disease (FD) include migalastat, an oral pharmacological chaperone, and agalsidase beta and agalsidase alfa, 2 forms of enzyme replacement therapy. Broad tissue distribution may be beneficial for clinical efficacy in FD,
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9b01bfaa1a10e76630531521152accfd
https://doi.org/10.1002/cpdd.941
https://doi.org/10.1002/cpdd.941
Autor:
Mouna Taroua, Christiane Auray-Blais, Siamak Jabbarzadeh-Tabrizi, Raphael Schiffmann, Jin-Song Shen, Taniqua S. Day, Michel Boutin
Publikováno v:
Journal of Lipid Research, Vol 61, Iss 11, Pp 1410-1423 (2020)
J Lipid Res
J Lipid Res
Fabry disease is caused by deficient activity of α-galactosidase A, an enzyme that hydrolyzes the terminal α-galactosyl moieties from glycolipids and glycoproteins, and subsequent accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0f9ccce41163000aa69e5598147f4f22
http://www.sciencedirect.com/science/article/pii/S0022227520437290
http://www.sciencedirect.com/science/article/pii/S0022227520437290