Better understanding the neurobiology of primary lateral sclerosis. (2nd November 2020)
- Record Type:
- Journal Article
- Title:
- Better understanding the neurobiology of primary lateral sclerosis. (2nd November 2020)
- Main Title:
- Better understanding the neurobiology of primary lateral sclerosis
- Authors:
- Ozdinler, P. Hande
Gautam, Mukesh
Gozutok, Oge
Konrad, Csaba
Manfredi, Giovanni
Area Gomez, Estela
Mitsumoto, Hiroshi
Erb, Marcella L.
Tian, Zheng
Haase, Georg - Abstract:
- Abstract: Primary lateral sclerosis (PLS) is a rare neurodegenerative disease characterized by progressive degeneration of upper motor neurons (UMNs). Recent studies shed new light onto the cellular events that are particularly important for UMN maintenance including intracellular trafficking, mitochondrial energy homeostasis and lipid metabolism. This review summarizes these advances including the role of Alsin as a gene linked to atypical forms of juvenile PLS, and discusses wider aspects of cellular pathology that have been observed in adult forms of PLS. The review further discusses the prospects of new transgenic upper motor neuron reporter mice, human stem cell-derived UMN cultures, cerebral organoids and non-human primates as future model systems to better understand and ultimately treat PLS.
- Is Part Of:
- Amyotrophic lateral sclerosis and frontotemporal degeneration. Volume 21(2020)Supplement 1
- Journal:
- Amyotrophic lateral sclerosis and frontotemporal degeneration
- Issue:
- Volume 21(2020)Supplement 1
- Issue Display:
- Volume 21, Issue 1 (2020)
- Year:
- 2020
- Volume:
- 21
- Issue:
- 1
- Issue Sort Value:
- 2020-0021-0001-0000
- Page Start:
- 35
- Page End:
- 46
- Publication Date:
- 2020-11-02
- Subjects:
- primary lateral sclerosis -- Betz cell -- upper motor neuron -- corticospinal motor neuron -- Alsin -- ALS2 -- endosomes -- mitochondria -- Golgi apparatus -- bioenergetics -- membrane lipids
616.839 - Journal URLs:
- http://informahealthcare.com/journal/afd ↗
http://informahealthcare.com ↗ - DOI:
- 10.1080/21678421.2020.1837175 ↗
- Languages:
- English
- ISSNs:
- 2167-8421
- Deposit Type:
- Legaldeposit
- View Content:
- Available online (eLD content is only available in our Reading Rooms) ↗
- Physical Locations:
- British Library DSC - 0859.841188
British Library DSC - BLDSS-3PM
British Library STI - ELD Digital store - Ingest File:
- 23573.xml