Zobrazeno 1 - 10
of 14
pro vyhledávání: '"Sara K. Custer"'
Autor:
Jonathan J Cherry, Christine J DiDonato, Elliot J Androphy, Alessandro Calo, Kyle Potter, Sara K Custer, Sarah Du, Timothy L Foley, Ariamala Gopalsamy, Emily J Reedich, Susana M Gordo, William Gordon, Natalie Hosea, Lyn H Jones, Daniel K Krizay, Gregory LaRosa, Hongxia Li, Sachin Mathur, Carol A Menard, Paraj Patel, Rebeca Ramos-Zayas, Anne Rietz, Haojing Rong, Baohong Zhang, Michael A Tones
Publikováno v:
PLoS ONE, Vol 12, Iss 9, p e0185079 (2017)
C5-substituted 2,4-diaminoquinazoline inhibitors of the decapping scavenger enzyme DcpS (DAQ-DcpSi) have been developed for the treatment of spinal muscular atrophy (SMA), which is caused by genetic deficiency in the Survival Motor Neuron (SMN) prote
Externí odkaz:
https://doaj.org/article/d509895517b840fca86c06c702f957d6
Publikováno v:
Neurobiol Aging
Alzheimer’s disease (AD) is the most common cause of dementia, afflicting more than 5 million Americans; it is the 6th leading cause of death in the United States. As the population ages, the number of Americans with AD expected to increase dramati
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::314f689480b203d5c4cac5cf041d18bb
https://doi.org/10.1016/j.neurobiolaging.2021.01.003
https://doi.org/10.1016/j.neurobiolaging.2021.01.003
Autor:
Sara K Custer, Timra D Gilson, Hongxia Li, A Gary Todd, Jacob W Astroski, Hai Lin, Yunlong Liu, Elliot J Androphy
Publikováno v:
PLoS ONE, Vol 11, Iss 10, p e0163954 (2016)
Spinal muscular atrophy (SMA) is an intractable neurodegenerative disease afflicting 1 in 6-10,000 live births. One of the key functions of the SMN protein is regulation of spliceosome assembly. Reduced levels of the SMN protein that are observed in
Externí odkaz:
https://doaj.org/article/bfc2693e13524baf93b0e8f238488ec0
Publikováno v:
Biochem Biophys Res Commun
We report that expression of the α-COP protein rescues disease phenotype in a severe mouse model of Spinal Muscular Atrophy (SMA).. Lentiviral particles expressing α-COP were injected directly into the testes of genetically pure mouse strain of int
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::98eaf858cb4bb66de2f392a6596338e5
https://doi.org/10.1016/j.bbrc.2019.04.176
https://doi.org/10.1016/j.bbrc.2019.04.176
Publikováno v:
Brain Research. 1706:135-146
We report here the finding of abnormal Golgi apparatus morphology in motor neuron like cells depleted of SMN as well as Golgi apparatus morphology in SMA patient fibroblasts. Rescue experiments demonstrate that this abnormality is dependent on SMN, b
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::43e1dc6c3d401391f646bb1fdf501e57
https://doi.org/10.1016/j.brainres.2018.11.005
https://doi.org/10.1016/j.brainres.2018.11.005
Autor:
Taylor Beahrs, Virginia M. Sanders, Sara K. Custer, Nicole D. Schartz, Deborah O. Setter, Melissa M. Haulcomb, Rena M. Meadows, Kathryn J. Jones
Publikováno v:
Restorative Neurology and Neuroscience. 36:417-422
BACKGROUND When nerve transection is performed on adult rodents, a substantial population of neurons survives short-term disconnection from target, and the immune system supports this neuronal survival, however long-term survival remains unknown. Und
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b711f74e76f63c497eefd44ed9f3f81a
https://doi.org/10.3233/rnn-170809
https://doi.org/10.3233/rnn-170809
Autor:
Timra Gilson, Le Thi Hao, Christine E. Beattie, Sara K. Custer, Elliot J. Androphy, Hongxia Li
Publikováno v:
Human Molecular Genetics. 24:7295-7307
Spinal muscular atrophy (SMA), a heritable neurodegenerative disease, results from insufficient levels of the survival motor neuron (SMN) protein. α-COP binds to SMN, linking the COPI vesicular transport pathway to SMA. Reduced levels of α-COP rest
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cbfe565cd0b5d3fe63a903e40d26246a
https://doi.org/10.1093/hmg/ddv428
https://doi.org/10.1093/hmg/ddv428
Autor:
Sachin Mathur, Jonathan J. Cherry, Baohong Zhang, Christine J. DiDonato, Rebeca Ramos-Zayas, Kyle Potter, Gregory LaRosa, Michael A. Tones, Haojing Rong, Natalie Hosea, Alessandro Calo, Timothy L. Foley, Sarah Du, William Gordon, Carol A. Menard, Paraj Patel, Susana Gordo, Elliot J. Androphy, Anne Rietz, Lyn H. Jones, Sara K. Custer, Daniel K. Krizay, Emily J. Reedich, Ariamala Gopalsamy, Hongxia Li
Publikováno v:
PLoS ONE, Vol 12, Iss 9, p e0185079 (2017)
PLoS ONE
PLoS ONE
C5-substituted 2,4-diaminoquinazoline inhibitors of the decapping scavenger enzyme DcpS (DAQ-DcpSi) have been developed for the treatment of spinal muscular atrophy (SMA), which is caused by genetic deficiency in the Survival Motor Neuron (SMN) prote
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::62a8004254f0368601d90fc794482243
http://europepmc.org/articles/PMC5612656?pdf=render
http://europepmc.org/articles/PMC5612656?pdf=render
Publikováno v:
Human Molecular Genetics. 19:1741-1755
Inclusion body myopathy associated with Paget's disease of bone and frontotemporal dementia (IBMPFD) is a dominantly inherited degenerative disorder caused by mutations in the valosin-containing protein (VCP) gene. VCP (p97 in mouse, TER94 in Drosoph
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f2e6cd3c0f0d9a7d28d2f2001c364ba7
https://doi.org/10.1093/hmg/ddq050
https://doi.org/10.1093/hmg/ddq050
Autor:
Sara K. Custer, Albert R. La Spada, Bryce L. Sopher, Udo Rueb, Thomas Deller, Christian Schultz, Randell T. Libby, Gwenn A. Garden, Lesnick E Westrum, Stephan J. Guyenet, Nishi Gill
Publikováno v:
Nature Neuroscience. 9:1302-1311
Non-neuronal cells may be pivotal in neurodegenerative disease, but the mechanistic basis of this effect remains ill-defined. In the polyglutamine disease spinocerebellar ataxia type 7 (SCA7), Purkinje cells undergo non-cell-autonomous degeneration i
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::668d5314242832629d7c4cc46176f63d
https://doi.org/10.1038/nn1750
https://doi.org/10.1038/nn1750