Granulins modulate liquid-liquid phase separation and aggregation of the prion-like C-terminal domain of the neurodegeneration-associated protein TDP-43.
ALS
ALS (Lou Gehrig disease)
FTLD
Granulins
TAR DNA-binding protein 43 (TDP-43, TARDBP)
intrinsically disordered protein
liquid–liquid phase separation
progranulin
protein dynamic
protein–protein interaction
stress granule
Journal
The Journal of biological chemistry
ISSN: 1083-351X
Titre abrégé: J Biol Chem
Pays: United States
ID NLM: 2985121R
Informations de publication
Date de publication:
21 02 2020
21 02 2020
Historique:
received:
15
10
2019
revised:
02
01
2020
pubmed:
9
1
2020
medline:
21
10
2020
entrez:
9
1
2020
Statut:
ppublish
Résumé
TAR DNA-binding protein 43 (TDP-43) has emerged as a key player in many neurodegenerative pathologies, including frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). Hallmarks of both FTLD and ALS are the toxic cytoplasmic inclusions of the prion-like C-terminal fragments of TDP-43 CTD (TDP-43 C-terminal domain), formed upon proteolytic cleavage of full-length TDP-43 in the nucleus and subsequent transport to the cytoplasm. Both full-length TDP-43 and its CTD are also known to form stress granules by coacervating with RNA in the cytoplasm during stress and may be involved in these pathologies. Furthermore, mutations in the
Identifiants
pubmed: 31911437
pii: S0021-9258(17)48284-6
doi: 10.1074/jbc.RA119.011501
pmc: PMC7039568
pii:
doi:
Substances chimiques
Benzothiazoles
0
DNA-Binding Proteins
0
Granulins
0
Prions
0
Protein Aggregates
0
thioflavin T
2390-54-7
RNA
63231-63-0
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, U.S. Gov't, Non-P.H.S.
Langues
eng
Sous-ensembles de citation
IM
Pagination
2506-2519Subventions
Organisme : NIGMS NIH HHS
ID : P20 GM103476
Pays : United States
Organisme : NIGMS NIH HHS
ID : R01 GM120634
Pays : United States
Organisme : NIA NIH HHS
ID : R56 AG062292
Pays : United States
Organisme : NIA NIH HHS
ID : RF1 AG055088
Pays : United States
Informations de copyright
© 2020 Bhopatkar et al.
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