Zobrazeno 1 - 10
of 21
pro vyhledávání: '"Omid Motabar"'
Autor:
Mylinh Vu, Rong Li, Amanda Baskfield, Billy Lu, Atena Farkhondeh, Kirill Gorshkov, Omid Motabar, Jeanette Beers, Guokai Chen, Jizhong Zou, Angela J. Espejo-Mojica, Alexander Rodríguez-López, Carlos J. Alméciga-Díaz, Luis A. Barrera, Xuntian Jiang, Daniel S. Ory, Juan J. Marugan, Wei Zheng
Publikováno v:
Orphanet Journal of Rare Diseases, Vol 13, Iss 1, Pp 1-15 (2018)
Abstract Background Tay-Sachs disease (TSD) is a rare neurodegenerative disorder caused by autosomal recessive mutations in the HEXA gene on chromosome 15 that encodes β-hexosaminidase. Deficiency in HEXA results in accumulation of GM2 ganglioside,
Externí odkaz:
https://doaj.org/article/bca708979a86458480f946c7d0be405e
Autor:
Ehud Goldin, Wei Zheng, Omid Motabar, Noel Southall, Jae Hyuk Choi, Juan Marugan, Christopher P Austin, Ellen Sidransky
Publikováno v:
PLoS ONE, Vol 7, Iss 1, p e29861 (2012)
Gaucher disease (GD), the most common lysosomal storage disorder, results from the inherited deficiency of the lysosomal enzyme glucocerebrosidase (GCase). Previously, wildtype GCase was used for high throughput screening (HTS) of large collections o
Externí odkaz:
https://doaj.org/article/1bc1b33deb1a40bbb50a4931ccbcfb4b
Autor:
Paul Shinn, Wei Sun, Miao Xu, Xiangguo Qiu, Gregory J. Tawa, Carles Martínez-Romero, Shu Yang, Ethan G. Fisher, Philip E. Sanderson, Omid Motabar, Yan Long, Wei Zheng, Shihua He, Jennifer Kouznetsova, Peter R. Williamson, Adolfo García-Sastre
Publikováno v:
Antiviral Research. 137:165-172
Although a group of FDA-approved drugs were previously identified with activity against Ebola virus (EBOV), most of them are not clinically useful because their human blood concentrations are not high enough to inhibit EBOV infection. We screened 795
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::91829f4a9b36e64831c01d150d9e1d2d
https://doi.org/10.1016/j.antiviral.2016.11.017
https://doi.org/10.1016/j.antiviral.2016.11.017
Publikováno v:
Annals of the New York Academy of Sciences. 1371:15-29
Lysosomal storage diseases (LSDs) are a group of rare diseases in which the function of the lysosome is disrupted by the accumulation of macromolecules. The complexity underlying the pathogenesis of LSDs and the small, often pediatric, population of
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::1c353a447d4eec301033a646fd88b068
https://doi.org/10.1111/nyas.13052
https://doi.org/10.1111/nyas.13052
Autor:
Wei Zheng, Daniel S. Ory, Omid Motabar, Xuntian Jiang, Rong Li, Carlos J. Alméciga-Díaz, Jizhong Zou, Amanda Baskfield, Guokai Chen, Luis A. Barrera, Alexander Rodríguez-López, Kirill Gorshkov, Mylinh Vu, Atena Farkhondeh, Angela J. Espejo-Mojica, Billy Lu, Juan J. Marugan, Jeanette Beers
Publikováno v:
Orphanet Journal of Rare Diseases, Vol 13, Iss 1, Pp 1-15 (2018)
Background Tay-Sachs disease (TSD) is a rare neurodegenerative disorder caused by autosomal recessive mutations in the HEXA gene on chromosome 15 that encodes β-hexosaminidase. Deficiency in HEXA results in accumulation of GM2 ganglioside, a glycosp
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d0a458f4d971b7bce449d6bb36e6240e
https://doi.org/10.1186/s13023-018-0886-3
https://doi.org/10.1186/s13023-018-0886-3
Autor:
Wei Zheng, Wendy Westbroek, Noel Southall, Ehud Goldin, Juan J. Marugan, Ellen Sidransky, Jae H. Choi, Samarjit Patnaik, Omid Motabar, William Leister, Arash Velayati, Wendy A. Lea
Publikováno v:
Journal of Medicinal Chemistry. 55:5734-5748
A major challenge in the field of Gaucher disease has been the development of new therapeutic strategies including molecular chaperones. All previously described chaperones of glucocerebrosidase are enzyme inhibitors, which complicates their clinical
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::bd304403c145327182e1dee08b78860e
https://doi.org/10.1021/jm300063b
https://doi.org/10.1021/jm300063b
Autor:
Ehud Goldin, Juan J. Marugan, Ellen Sidransky, Jingbo Xiao, Noel Southall, Wei Zheng, Samarjit Patnaik, Wendy A. Lea, Anton Simeonov, Omid Motabar, Maria DeBernardi, Wenwei Huang, Wendy Westbroek
Publikováno v:
Med. Chem. Commun.. 3:56-60
Small molecule chaperones are a promising therapeutic approach for the Lysosomal Storage Disorders (LSDs). Here, we report the discovery of a new series of non-iminosugar glucocerebrosidase inhibitors with chaperone capacity, and describe their struc
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::975a987811e608de710184d7c7aff0b0
https://doi.org/10.1039/c1md00200g
https://doi.org/10.1039/c1md00200g
Publikováno v:
Current Chemical Genomics
Fabry disease is a rare inherited lysosomal storage disorder caused by a partial or complete deficiency of α-galactosidase A (GLA), resulting in the storage of excess cellular glycosphingolipids. Enzyme replacement therapy is available for the treat
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::81be2396e0e507e7eda33a7ae06665a3
https://doi.org/10.2174/1875397301004010050
https://doi.org/10.2174/1875397301004010050
Autor:
Christopher P. Austin, Noel Southall, Ke Liu, Gary L. Griffiths, Ehud Goldin, Ellen Sidransky, Wei Zheng, Zhen-Dan Shi, Omid Motabar
Publikováno v:
Analytical Biochemistry. 390:79-84
Mutations in alpha-glucosidase cause accumulation of glycogen in lysosomes, resulting in Pompe disease, a lysosomal storage disorder. Small molecule chaperones that bind to enzyme proteins and correct the misfolding and mistrafficking of mutant prote
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b891b298d81fe7897646d1fdf3c2b10a
https://doi.org/10.1016/j.ab.2009.04.010
https://doi.org/10.1016/j.ab.2009.04.010
Publikováno v:
Annals of the New York Academy of Sciences. 1371(1)
Lysosomal storage diseases (LSDs) are a group of rare diseases in which the function of the lysosome is disrupted by the accumulation of macromolecules. The complexity underlying the pathogenesis of LSDs and the small, often pediatric, population of
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::d38cb23c0e3ff311e9e628c6abf10a45
https://pubmed.ncbi.nlm.nih.gov/27144735
https://pubmed.ncbi.nlm.nih.gov/27144735