Zobrazeno 1 - 10
of 41
pro vyhledávání: '"Sharmistha Mitra"'
Autor:
Sharmistha Mitra, Baozhi Chen, Peixiang Wang, Erin E. Chown, Mathew Dear, Dikran R. Guisso, Ummay Mariam, Jun Wu, Emrah Gumusgoz, Berge A. Minassian
Publikováno v:
Disease Models & Mechanisms, Vol 16, Iss 1 (2023)
Externí odkaz:
https://doaj.org/article/24f6a47e51664198bbbbbe98bb91751b
Publikováno v:
Rev Neurol (Paris)
The ubiquitin system impacts most cellular processes and is altered in numerous neurodegenerative diseases . However, little is known about its role in neurodegenerative diseases due to disturbances of glycogen metabolism such as Lafora disease (LD).
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f6584b5adb3e451b322f0ee9da392bca
https://doi.org/10.1016/j.neurol.2021.06.006
https://doi.org/10.1016/j.neurol.2021.06.006
Autor:
Silvia Nitschke, Mitchell A Sullivan, Sharmistha Mitra, Charlotte R Marchioni, Jennifer P Y Lee, Brandon H Smith, Saija Ahonen, Jun Wu, Erin E Chown, Peixiang Wang, Sara Petković, Xiaochu Zhao, Laura F DiGiovanni, Ami M Perri, Lori Israelian, Tamar R Grossman, Holly Kordasiewicz, Francisco Vilaplana, Kazuhiro Iwai, Felix Nitschke, Berge A Minassian
Publikováno v:
Brain
Longer glucan chains tend to precipitate. Glycogen, by far the largest mammalian glucan and the largest molecule in the cytosol with up to 55 000 glucoses, does not, due to a highly regularly branched spherical structure that allows it to be perfused
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f59293c4929b2bbb5d47f012bf06d7c1
https://doi.org/10.1093/brain/awac017
https://doi.org/10.1093/brain/awac017
Publikováno v:
Biochemical Society Transactions
Altered protein ubiquitination is associated with the pathobiology of numerous diseases; however, its involvement in glycogen metabolism and associated polyglucosan body (PB) disease has not been investigated in depth. In PB disease, excessively long
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::71d0d444d4a72ab54ff426a0cdcfcfca
http://europepmc.org/articles/PMC8589444
http://europepmc.org/articles/PMC8589444
Autor:
Dikran R Guisso, Silvia Nitschke, Berge A. Minassian, Jun Wu, Brandy Verhalen, Matthew Dear, Felix Nitschke, Emrah Gumusgoz, Sharmistha Mitra, Sahba Kasiri
Publikováno v:
Neurotherapeutics
Many adult and most childhood neurological diseases have a genetic basis. CRISPR/Cas9 biotechnology holds great promise in neurological therapy, pending the clearance of major delivery, efficiency, and specificity hurdles. We applied CRISPR/Cas9 geno
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::75671ca9d579993e6f911c9c05db849c
https://doi.org/10.1007/s13311-021-01040-7
https://doi.org/10.1007/s13311-021-01040-7
Publikováno v:
Nature Reviews Neurology. 14:606-617
Lafora disease is a severe, autosomal recessive, progressive myoclonus epilepsy. The disease usually manifests in previously healthy adolescents, and death commonly occurs within 10 years of symptom onset. Lafora disease is caused by loss-of-function
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::287267385c115113c1cc475d79fef448
https://doi.org/10.1038/s41582-018-0057-0
https://doi.org/10.1038/s41582-018-0057-0
Publikováno v:
Statistical Quality Technologies ISBN: 9783030207083
In this paper, we design a decision theoretic sampling plan (DSP) based on Type-I and Type-I hybrid censored lifetime data from a one-parameter exponential distribution. The Bayes estimator of the mean lifetime is used to define a decision function.
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::18e275bc1a5ec1a6595edaab9cb92d69
https://doi.org/10.1007/978-3-030-20709-0_8
https://doi.org/10.1007/978-3-030-20709-0_8
Autor:
Jason G. McCoy, Ming Zhou, Wonpil Im, Jumin Lee, Sébastien F. Poget, Zhenning Ren, Sharmistha Mitra, Elena J. Levin, Vitali Stanevich, Matthias Quick
Publikováno v:
Structure. 24:956-964
The phosphoenolpyruvate:carbohydrate phosphotransferase systems are found in bacteria, where they play central roles in sugar uptake and regulation of cellular uptake processes. Little is known about how the membrane-embedded components (EIICs) selec
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ae3d87109fd9546e3be6ee085d6b5682
https://doi.org/10.1016/j.str.2016.04.003
https://doi.org/10.1016/j.str.2016.04.003
The study proposes a new decision theoretic sampling plan (DSP) for Type-I and Type-I hybrid censored samples when the lifetimes of individual items are exponentially distributed with a scale parameter. The DSP is based on an estimator of the scale p
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f393bfca7bd8140bc164732abcbf8701
http://arxiv.org/abs/1807.00615
http://arxiv.org/abs/1807.00615
Autor:
Ami M. Perri, Saija Ahonen, Xiaochu Zhao, Lori Israelian, Felix Nitschke, Cameron Ackerley, Berge A. Minassian, Mitchell A. Sullivan, Sharmistha Mitra, Laura F. DiGiovanni, Erin E. Chown, Mackenzie Chown, Peixiang Wang
SUMMARYGlycogen synthesis is vital, malstructure resulting in precipitation and accumulation into neurotoxic polyglucosan bodies (PBs). One well-understood mechanism of PB generation is glycogen branching enzyme deficiency (GBED). Less understood is
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::de9c5e6cd2df8cccc6a673ffc341fb8f