Zobrazeno 1 - 9
of 9
pro vyhledávání: '"John H Wen"'
Publikováno v:
PLoS ONE, Vol 10, Iss 8, p e0135591 (2015)
Alpha-1 antitrypsin deficiency is the leading cause of childhood liver failure and one of the most common lethal genetic diseases. The disease-causing mutant A1AT-Z fails to fold correctly and accumulates in the endoplasmic reticulum (ER) of the live
Externí odkaz:
https://doaj.org/article/d55ee23894054f3682f195ba6a1b4d7a
Autor:
Lawrence S. Hsieh, John H. Wen, Kumiko Claycomb, Yuegao Huang, Felicia A. Harrsch, Janice R. Naegele, Fahmeed Hyder, Gordon F. Buchanan, Angelique Bordey
Publikováno v:
Nature Communications, Vol 7, Iss 1, Pp 1-12 (2016)
The etiology of focal cortical dysplasia (FCD) is not fully understood. Here authors generate an mTORC1 overactivation mouse model that recapitulates hallmarks of type II FCDs, including spontaneous seizures, and suggest that neuronal defects, rather
Externí odkaz:
https://doaj.org/article/f7ba5ffa0e6d426fa4bef37c114838b1
Autor:
Dennis D. Spencer, Stephanie A. Getz, Lawrence S. Hsieh, John H. Wen, Ying Wang, Longbo Zhang, Lena H. Nguyen, Juan Torres-Reveron, Angélique Bordey
Publikováno v:
Sci Transl Med
The causative link between focal cortical malformations (FCM) and epilepsy is well-accepted, especially among patients with focal cortical dysplasia type II (FCDII) and tuberous sclerosis complex (TSC). However, the mechanisms underlying seizures rem
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0a0997c308fb97a8f0e60782e4bad719
https://doi.org/10.1126/scitranslmed.abc1492
https://doi.org/10.1126/scitranslmed.abc1492
Autor:
Lawrence S. Hsieh, Dennis D. Spencer, Lena H. Nguyen, John H. Wen, Longbo Zhang, Angélique Bordey, Juan Torres-Reveron
The causative link between focal cortical malformations (FCM) and epilepsy is well-accepted, especially among patients with focal cortical dysplasia type II (FCDII) and tuberous sclerosis complex (TSC). However, the mechanisms underlying seizures rem
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9470a7c8221d842be0c7f8b670fd6f1e
https://doi.org/10.1101/853820
https://doi.org/10.1101/853820
Publikováno v:
Neuroscience Letters. 637:142-147
Inbred mouse strains have been used preferentially for behavioral testing over outbred counterparts, even though outbred mice reflect the genetic diversity in the human population better. Here, we compare the sociability of widely available outbred C
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::679a160c4eba912443a8c09e7dd9e23f
https://doi.org/10.1016/j.neulet.2016.11.035
https://doi.org/10.1016/j.neulet.2016.11.035
Autor:
John H. Wen, Angélique Bordey, Tiffany V. Lin, Longbo Zhang, Xuan Gong, Lawrence S. Hsieh, Laura Miyares, Tianxiang Huang
Publikováno v:
Human Molecular Genetics. 24:5746-5758
Abnormal axonal connectivity and hyperactive mTOR complex 1 (mTORC1) are shared features of several neurological disorders. Hyperactive mTORC1 alters axon length and polarity of hippocampal neurons in vitro, but the impact of hyperactive mTORC1 on ax
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::144b5b7980a61ca234ff410231272daf
https://doi.org/10.1093/hmg/ddv295
https://doi.org/10.1093/hmg/ddv295
Autor:
Gordon F. Buchanan, Felicia A. Harrsch, Yuegao Huang, Lawrence S. Hsieh, Kumiko Claycomb, Fahmeed Hyder, John H. Wen, Angélique Bordey, Janice R. Naegele
Publikováno v:
Nature Communications
Nature Communications, Vol 7, Iss 1, Pp 1-12 (2016)
Nature Communications, Vol 7, Iss 1, Pp 1-12 (2016)
Focal cortical dysplasia (FCD), a local malformation of cortical development, is the most common cause of pharmacoresistant epilepsy associated with life-long neurocognitive impairments. It remains unclear whether neuronal misplacement is required fo
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::573a18d934baa50744e74197bb382b68
https://doi.org/10.1038/ncomms11753
https://doi.org/10.1038/ncomms11753
Publikováno v:
PLoS ONE, Vol 10, Iss 8, p e0135591 (2015)
PLoS ONE
PLoS ONE
Alpha-1 antitrypsin deficiency is the leading cause of childhood liver failure and one of the most common lethal genetic diseases. The disease-causing mutant A1AT-Z fails to fold correctly and accumulates in the endoplasmic reticulum (ER) of the live
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f3bfed8c2d9314f8f969686f49f57377
http://europepmc.org/articles/PMC4546667?pdf=render
http://europepmc.org/articles/PMC4546667?pdf=render
Publikováno v:
The FASEB Journal. 28
Alpha-1 antitrypsin deficiency is the leading cause of childhood liver failure, one of the most common lethal genetic diseases, and is present in 1 in 2,500 people of European descent. The disease-causing mutant A1AT-Z fails to fold correctly and acc
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::653baf7e9c0ae72df29e6ca42ffc2ea1
https://doi.org/10.1096/fasebj.28.1_supplement.949.2
https://doi.org/10.1096/fasebj.28.1_supplement.949.2