Zobrazeno 1 - 10
of 37
pro vyhledávání: '"Joshua T Dearborn"'
Autor:
David F Wozniak, Kelly A Diggs-Andrews, Sara Conyers, Carla M Yuede, Joshua T Dearborn, Jacquelyn A Brown, Kazuhiro Tokuda, Yukitoshi Izumi, Charles F Zorumski, David H Gutmann
Publikováno v:
PLoS ONE, Vol 8, Iss 6, p e66024 (2013)
Children with neurofibromatosis type 1 (NF1) frequently have cognitive and behavioral deficits. Some of these deficits have been successfully modeled in Nf1 genetically-engineered mice that develop optic gliomas (Nf1 OPG mice). In the current study,
Externí odkaz:
https://doaj.org/article/53ca7f9a2d8f4fef890d35d461b66817
Autor:
Chihiro Sato, Mustafa Turkoz, Joshua T Dearborn, David F Wozniak, Raphael Kopan, Matthew R Hass
Publikováno v:
PLoS ONE, Vol 7, Iss 10, p e48180 (2012)
Previous studies suggest that loss of γ-secretase activity in postnatal mouse brains causes age-dependent memory impairment and neurodegeneration. Due to the diverse array of γ-secretase substrates, it remains to be demonstrated whether loss of cle
Externí odkaz:
https://doaj.org/article/866e48f701074e758bca789c28719da6
Autor:
Joshua T. Dearborn, Hemanth R. Nelvagal, Nicholas R. Rensing, Keigo Takahashi, Stephanie M. Hughes, Thomas M. Wishart, Jonathan D. Cooper, Michael Wong, Mark S. Sands
Publikováno v:
Scientific Reports, Vol 12, Iss 1, Pp 1-13 (2022)
Abstract Cannabidiol (CBD) has gained attention as a therapeutic agent and is purported to have immunomodulatory, neuroprotective, and anti-seizure effects. Here, we determined the effects of chronic CBD administration in a mouse model of CLN1 diseas
Externí odkaz:
https://doaj.org/article/350eddea052f435687b37a8c0940ae75
Autor:
Keigo Takahashi, Elizabeth M. Eultgen, Sophie H. Wang, Nicholas R. Rensing, Hemanth R. Nelvagal, Joshua T. Dearborn, Olivier Danos, Nicholas Buss, Mark S. Sands, Michael Wong, Jonathan D. Cooper
Publikováno v:
The Journal of Clinical Investigation, Vol 133, Iss 12 (2023)
Although a disease-modifying therapy for classic late infantile neuronal ceroid lipofuscinosis (CLN2 disease) exists, poor understanding of cellular pathophysiology has hampered the development of more effective and persistent therapies. Here, we inv
Externí odkaz:
https://doaj.org/article/5aed6f53cf974a23b8c8e656557757e8
Autor:
Hemanth R. Nelvagal, Samantha L. Eaton, Sophie H. Wang, Elizabeth M. Eultgen, Keigo Takahashi, Steven Q. Le, Rachel Nesbitt, Joshua T. Dearborn, Nicholas Siano, Ana C. Puhl, Patricia I. Dickson, Gerard Thompson, Fraser Murdoch, Paul M. Brennan, Mark Gray, Stephen N. Greenhalgh, Peter Tennant, Rachael Gregson, Eddie Clutton, James Nixon, Chris Proudfoot, Stefano Guido, Simon G. Lillico, C. Bruce A. Whitelaw, Jui-Yun Lu, Sandra L. Hofmann, Sean Ekins, Mark S. Sands, Thomas M. Wishart, Jonathan D. Cooper
Publikováno v:
The Journal of Clinical Investigation, Vol 132, Iss 20 (2022)
CLN1 disease, also called infantile neuronal ceroid lipofuscinosis (NCL) or infantile Batten disease, is a fatal neurodegenerative lysosomal storage disorder resulting from mutations in the CLN1 gene encoding the soluble lysosomal enzyme palmitoyl-pr
Externí odkaz:
https://doaj.org/article/fb7adb2cd6174d5a9c38555ece25def7
Autor:
Mark S. Sands, Jonathan D. Cooper, Joshua T. Dearborn, John R. Østergaard, Hemanth R. Nelvagal
Publikováno v:
Neuropathology and Applied Neurobiology
Nelvagal, H R, Dearborn, J T, Ostergaard, J R, Sands, M S & Cooper, J D 2021, ' Spinal manifestations of CLN1 disease start during the early postnatal period ', Neuropathology and Applied Neurobiology, vol. 47, no. 2, pp. 251-267 . https://doi.org/10.1111/nan.12658
Nelvagal, H R, Dearborn, J T, Ostergaard, J R, Sands, M S & Cooper, J D 2021, ' Spinal manifestations of CLN1 disease start during the early postnatal period ', Neuropathology and Applied Neurobiology, vol. 47, no. 2, pp. 251-267 . https://doi.org/10.1111/nan.12658
The spinal cord appears especially vulnerable in CLN1 disease, a fatal inherited lysosomal storage disorder. Our data reveal a much earlier onset of spinal cord disease and accompanying behavioural changes in CLN1 mice, while spinal maturation is sti
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4231ac8969d9dea8d88d72974cdaedcf
http://europepmc.org/articles/PMC7867600
http://europepmc.org/articles/PMC7867600
Autor:
Keigo Takahashi, Elizabeth M. Eultgen, Sophie H. Wang, Nicholas R. Rensing, Hemanth R. Nelvagal, Joshua T. Dearborn, Mark S. Sands, Michael Wong, Jonathan D. Cooper
AimsCLN2 disease is a fatal inherited childhood neurodegenerative disorder. Although a disease-modifying therapy now exists, a fundamental lack of understanding of disease pathogenesis has hampered development of more effective therapies. To better u
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::89ead0e11d68c769e07c390f5eb90b29
https://doi.org/10.1101/2022.03.11.483984
https://doi.org/10.1101/2022.03.11.483984
Autor:
Keigo Takahashi, Elizabeth M. Eultgen, Sophie H. Wang, Nicholas Rensing, Hemanth R. Nelvagal, Joshua T. Dearborn, Olivier Danos, Nicholas Buss, Mark Sands, Michael Wong, Jonathan D. Cooper
Publikováno v:
Molecular Genetics and Metabolism. 138:107333
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::f0509ab0c41fc8c52fc1e84c0bb4aea6
https://doi.org/10.1016/j.ymgme.2022.107333
https://doi.org/10.1016/j.ymgme.2022.107333
Autor:
Steven Q. Le, Shih-hsin Kan, Marie S. Roberts, Joshua T. Dearborn, Feng Wang, Shan Li, Elizabeth M. Snella, Jackie K. Jens, Bethann N. Valentine, Hemanth R. Nelvagal, Alexander Sorensen, Keerthana Chintalapati, Kevin Ohlemiller, Carole Vogler, Jonathan D. Cooper, Tsui-Fen Chou, N. Matthew Ellinwood, Jodi D. Smith, Mark S. Sands, Patricia I. Dickson
Recombinant human alpha-N-acetylglucosaminidase-insulin-like growth factor-2 (rhNAGLU-IGF2) is an investigational enzyme replacement therapy for Sanfilippo B, a lysosomal storage disease. Because recombinant human NAGLU (rhNAGLU) is poorly mannose 6-
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::cc5b2a458a55f3ed2b94ffc54f9ef5e1
https://doi.org/10.1101/2021.08.06.455469
https://doi.org/10.1101/2021.08.06.455469
Publikováno v:
Scientific Reports, Vol 9, Iss 1, Pp 1-14 (2019)
Scientific Reports
Scientific Reports
Here we have characterized the functional impairments resulting from conditional knockout of the ubiquitin-conjugating E2 enzyme (UBC13) in rodent cerebellar granule neurons, which greatly increases the parallel fiber presynaptic boutons and function
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::08101e05f05087bf6c9005b717ccb7fc
http://link.springer.com/article/10.1038/s41598-019-40714-3
http://link.springer.com/article/10.1038/s41598-019-40714-3