J
Jaime Hubbard
Researcher at Mayo Clinic
Publications - 3
Citations - 463
Jaime Hubbard is an academic researcher from Mayo Clinic. The author has contributed to research in topics: Transgene & Downregulation and upregulation. The author has an hindex of 3, co-authored 3 publications receiving 398 citations.
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Journal ArticleDOI
Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress
Yong Jie Zhang,Karen Jansen-West,Ya Fei Xu,Tania F. Gendron,Kevin F. Bieniek,Wen Lang Lin,Hiroki Sasaguri,Thomas R. Caulfield,Jaime Hubbard,Lillian M. Daughrity,Jeannie Chew,Veronique V. Belzil,Mercedes Prudencio,Jeannette N. Stankowski,Monica Castanedes-Casey,Ena C. Whitelaw,Peter E.A. Ash,Michael DeTure,Rosa Rademakers,Kevin B. Boylan,Dennis W. Dickson,Leonard Petrucelli +21 more
TL;DR: It is demonstrated that poly(GA) c9RAN proteins are neurotoxic and may be implicated in the neurodegenerative processes of c9FTD/ALS, and suggest that targeting the ER using small molecules may be a promising therapeutic approach for these devastating diseases.
Journal ArticleDOI
The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation
Yong Jie Zhang,Thomas R. Caulfield,Ya Fei Xu,Tania F. Gendron,Jaime Hubbard,Caroline Stetler,Hiroki Sasaguri,Ena C. Whitelaw,Shuyi Cai,Wing C. Lee,Leonard Petrucelli +10 more
TL;DR: New insight is provided into the structural basis for TAR DNA-binding protein-43 function and aggregation, and it is suggested that stabilization of TDP-43 homodimers, the physiologically active form of T DP-43, may be a promising therapeutic strategy for ALS and FTLD-TDP.
Journal ArticleDOI
The pathological phenotypes of human TDP-43 transgenic mouse models are independent of downregulation of mouse Tdp-43.
Ya Fei Xu,Mercedes Prudencio,Jaime Hubbard,Jimei Tong,Ena C. Whitelaw,Karen Jansen-West,Caroline Stetler,Xiangkun Cao,John Song,Yong Jie Zhang +9 more
TL;DR: It is observed that reduction of mTdp-43 in non-transgenic mice by intraventricular brain injection of AAV1-shTardbp leads to a dramatic increase in the levels of splicing variants of mouse sortilin 1 and translin, but these levels are not increased in hTDP-43 transgenic mice despite significant downregulation of m Tdp- 43 in these mice.