TDP-43 proteinopathy occurs independently of autophagic substrate accumulation and underlies nuclear defects in Niemann-Pick C disease.

Liu, Elaine A; Mori, Erika; Hamasaki, Fuko; Lieberman, Andrew P

Liu, Elaine A; Mori, Erika; Hamasaki, Fuko; Lieberman, Andrew P.

Affiliation

Liu EA; Department of Pathology, University of Michigan Medical School, Ann Arbor, MI, USA.
Mori E; Cellular and Molecular Biology Graduate Program, University of Michigan Medical School, Ann Arbor, MI, USA.
Hamasaki F; Medical Scientist Training Program, University of Michigan Medical School, Ann Arbor, MI, USA.
Lieberman AP; Yamaguchi University School of Medicine, Ube, Japan.

Neuropathol Appl Neurobiol ; 47(7): 1019-1032, 2021 12.

Article in En | MEDLINE | ID: mdl-34048071

Subject(s)

Autophagy/genetics; DNA-Binding Proteins/metabolism; Niemann-Pick Disease, Type C/genetics; Niemann-Pick Disease, Type C/pathology; Animals; Brain/pathology; DNA-Binding Proteins/genetics; Intracellular Signaling Peptides and Proteins/metabolism; Lysosomes/metabolism; Mice; Neuroinflammatory Diseases/genetics; Neuroinflammatory Diseases/metabolism; Neuroinflammatory Diseases/pathology; Neurons/pathology; Niemann-Pick Disease, Type C/metabolism; TDP-43 Proteinopathies/genetics; TDP-43 Proteinopathies/metabolism

Key words

Niemann-Pick type C; TDP-43; autophagy; lysosomal diseases; nucleocytoplasmic transport

Fulltext

Add to My VHL

XML

PubMed Links

Search on Google

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Autophagy / DNA-Binding Proteins / Niemann-Pick Disease, Type C Limits: Animals Language: En Journal: Neuropathol Appl Neurobiol Year: 2021 Document type: Article Affiliation country: United States Country of publication: United kingdom

Fulltext

Add to My VHL

XML

PubMed Links

Search on Google