Disease Models & Mechanisms (Dec 2022)
Lysosomal alterations and decreased electrophysiological activity in CLN3 disease patient-derived cortical neurons
- Sueanne Chear,
- Sharn Perry,
- Richard Wilson,
- Aidan Bindoff,
- Jana Talbot,
- Tyson L. Ware,
- Alexandra Grubman,
- James C. Vickers,
- Alice Pébay,
- Jonathan B. Ruddle,
- Anna E. King,
- Alex W. Hewitt,
- Anthony L. Cook
Affiliations
- Sueanne Chear
- Wicking Dementia Research and Education Centre, University of Tasmania, Hobart, TAS 7001, Australia
- Sharn Perry
- Wicking Dementia Research and Education Centre, University of Tasmania, Hobart, TAS 7001, Australia
- Richard Wilson
- Central Science Laboratory, University of Tasmania, Hobart, TAS 7001, Australia
- Aidan Bindoff
- Wicking Dementia Research and Education Centre, University of Tasmania, Hobart, TAS 7001, Australia
- Jana Talbot
- Wicking Dementia Research and Education Centre, University of Tasmania, Hobart, TAS 7001, Australia
- Tyson L. Ware
- Department of Paediatrics, Royal Hobart Hospital, Hobart, TAS 7000, Australia
- Alexandra Grubman
- Department of Anatomy and Developmental Biology, Monash University, Clayton, VIC 3800, Australia
- James C. Vickers
- Wicking Dementia Research and Education Centre, University of Tasmania, Hobart, TAS 7001, Australia
- Alice Pébay
- Department of Anatomy and Physiology, University of Melbourne, Parkville, VIC 3010, Australia
- Jonathan B. Ruddle
- Centre for Eye Research Australia, Royal Victorian Eye and Ear Hospital, East Melbourne, VIC 3002, Australia
- Anna E. King
- Wicking Dementia Research and Education Centre, University of Tasmania, Hobart, TAS 7001, Australia
- Alex W. Hewitt
- Menzies Institute for Medical Research, University of Tasmania, Hobart, TAS 7001, Australia
- Anthony L. Cook
- Wicking Dementia Research and Education Centre, University of Tasmania, Hobart, TAS 7001, Australia
- DOI
- https://doi.org/10.1242/dmm.049651
- Journal volume & issue
-
Vol. 15,
no. 12
Abstract
No abstracts available.Keywords
- juvenile neuronal ceroid lipofuscinosis
- batten disease
- dementia
- induced pluripotent stem cells
- microelectrode array
- proteomics