Zobrazeno 1 - 10
of 27
pro vyhledávání: '"Ronggen Fu"'
Publikováno v:
The Journal of Physiology. 589:2245-2260
Spinal motoneurons are highly vulnerable in amyotrophic lateral sclerosis (ALS). Previous research using a standard animal model, the mutant superoxide dismutase-1 (SOD1) mouse, has revealed deficits in many cellular properties throughout its lifespa
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::d4d0de073e0ed31d9ea1b0c948f4ce3f
https://doi.org/10.1113/jphysiol.2010.200659
https://doi.org/10.1113/jphysiol.2010.200659
Autor:
Jianxun Yi, Erdong Liu, Han Xiang Deng, Teepu Siddique, Eduardo Ríos, Jingsong Zhou, Ronggen Fu
Publikováno v:
Journal of Biological Chemistry. 285:705-712
Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disorder characterized by degeneration of motor neurons and atrophy of skeletal muscle. Mutations in the superoxide dismutase (SOD1) gene are linked to 20% cases of inherited ALS. Mitochond
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2238e3db5832675dec8a574f70675504
https://doi.org/10.1074/jbc.m109.041319
https://doi.org/10.1074/jbc.m109.041319
Publikováno v:
The Journal of Neuroscience. 29:15031-15038
Amyotrophic lateral sclerosis (ALS) is characterized by progressive degeneration of motoneurons. One potential mechanism is excitotoxicity. We studied the behaviors of spinal neurons using anin vitropreparation of the sacral cord from the G93A SOD1 m
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9051e17ef143d27a939b8180b10063ee
https://doi.org/10.1523/jneurosci.0574-09.2009
https://doi.org/10.1523/jneurosci.0574-09.2009
Publikováno v:
Proceedings of the National Academy of Sciences. 103:7148-7153
Point mutations in Cu, Zn-superoxide dismutase (SOD1) cause a familial form of the neurodegenerative disease amyotrophic lateral sclerosis (ALS). Aggregates of mutant SOD1 proteins are observed in histopathology and are invoked in several proposed me
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cd35fcfbefd38b2dfee7089657c5559a
https://doi.org/10.1073/pnas.0602048103
https://doi.org/10.1073/pnas.0602048103
Publikováno v:
The Journal of Physiology. 563:843-854
Mutations in the enzyme superoxide dismutase 1 (SOD1) initiate a progressive motoneurone degeneration in amyotrophic lateral sclerosis (ALS). Transgenic mice overexpressing this mutation develop a similar progressive motoneurone degeneration. In spin
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::58873b1c12e8793d03186a1fbd1c0e41
https://doi.org/10.1113/jphysiol.2004.074138
https://doi.org/10.1113/jphysiol.2004.074138
Autor:
George Gorrie, Ronggen Fu, Teepu Siddique, Erdong Liu, Sisi Li, Hong Zhai, Eileen H. Bigio, Faisal Fecto, Marco Martina, Daniel Radzicki, Craig Weiss, John F. Disterhoft, Hongxin Dong, Yong Shi, Hasan Arrat, Han Xiang Deng, Enrico Mugnaini
Publikováno v:
Proceedings of the National Academy of Sciences of the United States of America. 111(40)
Mutations in the gene encoding ubiquilin2 (UBQLN2) cause amyotrophic lateral sclerosis (ALS), frontotemporal type of dementia, or both. However, the molecular mechanisms are unknown. Here, we show that ALS/dementia-linked UBQLN2(P497H) transgenic mic
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::86fdcd80e48d4d7d45b2768b32c91346
https://pubmed.ncbi.nlm.nih.gov/25246588
https://pubmed.ncbi.nlm.nih.gov/25246588
Publikováno v:
Journal of neurophysiology. 107(1)
Riluzole is the only FDA-approved drug to treat amyotrophic lateral sclerosis, but its long-term effects on motoneurons are unknown. Therefore, we treated primary mouse spinal cord cultures with 2 μM riluzole for 4–9 days and then used whole cell
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ae02cef5dd1ec0008f2c814e976fe5ac
https://pubmed.ncbi.nlm.nih.gov/22013234
https://pubmed.ncbi.nlm.nih.gov/22013234
Autor:
Han-Xiang, Deng, Deng, Han-Xiang, Hujun, Jiang, Jiang, Hujun, Ronggen, Fu, Fu, Ronggen, Hong, Zhai, Zhai, Hong, Yong, Shi, Shi, Yong, Erdong, Liu, Liu, Erdong, Makito, Hirano, Hirano, Makito, Mauro C, Dal Canto, C Dal Canto, Mauro, Teepu, Siddique, Siddique, Teepu
Publikováno v:
Human Molecular Genetics
Mutations in Cu,Zn superoxide dismutase (SOD1) are associated with amyotrophic lateral sclerosis (ALS). Among more than 100 ALS-associated SOD1 mutations, premature termination codon (PTC) mutations exclusively occur in exon 5, the last exon of SOD1.
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ee2fef4c0d477fb853ff338a0e46c9e6
https://europepmc.org/articles/PMC2766822/
https://europepmc.org/articles/PMC2766822/
Autor:
Erdong Liu, Han Xiang Deng, Yi Yang, George Gorrie, Ronggen Fu, Teepu Siddique, Mauro C. Dal Canto, Enrico Mugnaini, Yong Shi, Hong Zhai
Publikováno v:
Human molecular genetics. 16(23)
Mutations in Alsin are associated with chronic juvenile amyotrophic lateral sclerosis (ALS2), juvenile primary lateral sclerosis and infantile-onset ascending spastic paralysis. The primary pathology and pathogenic mechanism of the disease remain lar
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::bdfd6e7f08b011fe4aa26354144b172d
https://pubmed.ncbi.nlm.nih.gov/17855450
https://pubmed.ncbi.nlm.nih.gov/17855450
Autor:
Erdong Liu, Hong Zhai, Han Xiang Deng, Mohammad S. Khan, Wu Yen Hung, Eileen H. Bigio, Ronggen Fu, Yong Shi, Teepu Siddique, George Gorrie, Thomas V. O'Halloran, Yoshiaki Furukawa, Thomas J. Lukas, Mauro C. Dal Canto
Twenty percent of the familial form of amyotrophic lateral sclerosis (ALS) is caused by mutations in the Cu, Zn-superoxide dismutase gene ( SOD1 ) through the gain of a toxic function. The nature of this toxic function of mutant SOD1 has remained lar
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::82278c0edf4f23de24007410555d0266
https://europepmc.org/articles/PMC1447523/
https://europepmc.org/articles/PMC1447523/