Disruption of ER-mitochondria signalling in fronto-temporal dementia and related amyotrophic lateral sclerosis.
Cell Death Dis
; 9(3): 327, 2018 02 28.
Article
en En
| MEDLINE
| ID: mdl-29491392
ABSTRACT
Fronto-temporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are two related and incurable neurodegenerative diseases. Features of these diseases include pathological protein inclusions in affected neurons with TAR DNA-binding protein 43 (TDP-43), dipeptide repeat proteins derived from the C9ORF72 gene, and fused in sarcoma (FUS) representing major constituent proteins in these inclusions. Mutations in C9ORF72 and the genes encoding TDP-43 and FUS cause familial forms of FTD/ALS which provides evidence to link the pathology and genetics of these diseases. A large number of seemingly disparate physiological functions are damaged in FTD/ALS. However, many of these damaged functions are regulated by signalling between the endoplasmic reticulum and mitochondria, and this has stimulated investigations into the role of endoplasmic reticulum-mitochondria signalling in FTD/ALS disease processes. Here, we review progress on this topic.
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Demencia
/
Retículo Endoplásmico
/
Esclerosis Amiotrófica Lateral
/
Mitocondrias
Límite:
Animals
/
Humans
Idioma:
En
Revista:
Cell Death Dis
Año:
2018
Tipo del documento:
Article
País de afiliación:
Reino Unido