Article

Defective cyclophilin A induces TDP-43 proteinopathy: implications for amyotrophic lateral sclerosis and frontotemporal dementia

2020-06-08

Abstract excerpt

Aggregation and cytoplasmic mislocalization of TDP-43 are pathological hallmarks of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) spectrum. However, the molecular mechanism by which TDP-43 aggregates form and cause neurodegeneration remains poorly understood. Cyclophilin A, also known as peptidyl-prolyl cis-trans isomerase A (PPIA), is a foldase and molecular chaperone. We previously found...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: 4bb43a94-0e4a-56e7-8fb9-12bb3324addd
DOI: 10.1101/2020.06.08.129528

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Defective cyclophilin A induces TDP-43 proteinopathy: implications for amyotrophic lateral sclerosis and frontotemporal dementiaDOI 10.1101/2020.06.08.129528Read

Peptidylprolyl isomerase A governs TARDBP function and assembly in heterogeneous nuclear ribonucleoprotein complexesSemantic proximity Read

TDP-43 aggregation mirrors TDP-43 knockdown, affecting the expression levels of a common set of proteinsSemantic proximity Read

Mitigation of TDP-43 toxic phenotype by an RGNEF fragment in amyotrophic lateral sclerosis models.Semantic proximity Read

Mitigation of TDP-43-induced toxic phenotype by expression of RGNEF N-terminal fragment in ALS modelsSemantic proximity Read

Differential loss of prolyl isomerase or chaperone activity of Ran-binding protein 2 (Ranbp2) unveils distinct physiological roles of its cyclophilin domain in proteostasis.Semantic proximity Read

Senataxin mutations elicit motor neuron degeneration phenotypes and yield TDP-43 mislocalization in ALS4 mice and human patients.Semantic proximity Read

Dysregulated actin dynamics and cofilin correlate with TDP-43 pathology in sporadic amyotrophic lateral sclerosisSemantic proximity Read

Cyclophilin A: a key player for human diseaseSemantic proximity Read

Accumulation of TDP-43 causes karyopherin-α4 pathology that characterises amyotrophic lateral sclerosisSemantic proximity Read

Proteostatic remodeling of small heat shock chaperones - crystallins by Ran-binding protein 2 and the peptidyl-prolyl <i>cis-trans</i> isomerase and chaperone activities of its cyclophilin domainSemantic proximity Read

TDP-43 knockdown in mouse model of ALS leads to dsRNA deposition, gliosis, and neurodegeneration in the spinal cordSemantic proximity Read

Early retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLD.Semantic proximity Read