Nuclear import receptors are recruited by FG-nucleoporins to rescue hallmarks of TDP-43 proteinopathy
Abstract
Cytoplasmic mislocalization and aggregation of TAR DNA-binding protein-43 (TDP-43) is a hallmark of the amyotrophic lateral sclerosis and frontotemporal dementia (ALS/FTD) disease spectrum, causing both nuclear loss-of-function and cytoplasmic toxic gain-of-function phenotypes. While TDP-43 proteinopathy has been associated with defects in nucleocytoplasmic transport, this process is still poorly understood. Here we study the role of karyopherin-β1 (KPNB1) and other nuclear import receptors in regulating TDP-43 pathology.
Document Details
- Document Type
- Pub Defense Publication
- Publication Date
- Dec 08, 2022
- Source ID
- 10.1186/s13024-022-00585-1
Entities
People
- Amanda M. Gleixner
- Bilal Khalil
- Björn Oskarsson
- Cara L. Croft
- Chih-wei Tsai
- Ching-chieh Chou
- Christian Puttinger
- Christopher J. Donnelly
- Chun Kim Lim
- Courtney L. Smith
- Deepak Chhangani
- Dennis W. Dickson
- Diego E. Rincon-limas
- Dmytro Morderer
- Feilin Liu
- Gael Fortin
- James Shorter
- Jannifer H. Lee
- Jingjiao Chen
- Junli Gao
- Ke Zhang
- Leonard Petrucelli
- Melissa C. Wren
- Sami J. Barmada
- Shige H. Yoshimura
- Todd E. Golde
- Wilfried Rossoll
- Xingli Li
Organizations
- BrightFocus Foundation
- Center for Scientific Review
- National Institutes of Health
- Rainwater Charitable Foundation
- Target ALS
- United States Department of Defense