Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

The J Domain of Sacsin Disrupts Intermediate Filament Assembly.

International journal of molecular sciences - 12 Dec 2022

Dabbaghizadeh Afrooz, Paré Alexandre, Cheng-Boivin Zacharie, Dagher Robin, Minotti Sandra, Dicaire Marie-Josée, Brais Bernard, Young Jason C, Durham Heather D, Gentil Benoit J

Abstract excerpt

Autosomal Recessive Spastic Ataxia of the Charlevoix Saguenay (ARSACS) is caused by mutation in the SACS gene resulting in loss of function of the protein sacsin. A key feature is the formation of abnormal bundles of neurofilaments (NF) in neurons and vimentin intermediate filaments (IF) in cultured fibroblasts, suggesting a role of sacsin in IF homeostasis. Sacsin contains a J domain (SacsJ) homologous to Hsp40,...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Heat-Shock Proteins
Share to Intermediate Filaments

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Motor Neurons

Share to Muscle Spasticity

Share to Mutation

Share to Spinocerebellar Ataxias

Share to Vimentin

Source

Open in PubMed Open full text

Identifiers

PMID: 36555380
DOI: 10.3390/ijms232415742
PMCID: PMC9779362

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

The J Domain of Sacsin Disrupts Intermediate Filament Assembly.PMID 36555380Read ↗

HDAC inhibitor SAHA resolved intermediate filament bundling and defective autophagy caused by the lack of sacsin in ARSACS.Semantic proximity Read ↗

Insights into SACS pathological attributes in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS)☆.Semantic proximity Read ↗

Altered organization of the intermediate filament cytoskeleton and relocalization of proteostasis modulators in cells lacking the ataxia protein sacsinSemantic proximity Read ↗

Subcellular, biochemical and biophysical alterations in two glial cell models of ARSACSSemantic proximity Read ↗

CK1α, FAM83H, and FAM83B contribute to bundling of neurofilaments and are sequestered in cellular and mice models of ARSACSSemantic proximity Read ↗

Comparative analysis and functional mapping of SACS mutations reveal novel insights into sacsin repeated architecture.Semantic proximity Read ↗

The ARSACS disease protein sacsin controls lysosomal positioning and reformation by regulating microtubule dynamics.Semantic proximity Read ↗

Mitochondrial dysfunction and Purkinje cell loss in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS)Semantic proximity Read ↗

Multi-omic profiling reveals the ataxia protein sacsin is required for integrin trafficking and synaptic organization.Semantic proximity Read ↗

A newly identified NES sequence present in spastin regulates its subcellular localization and microtubule severing activity.Semantic proximity Read ↗

Cytosolic sequestration of spatacsin by Protein Kinase A and 14-3-3 proteins.Semantic proximity Read ↗

Driving mitochondrial fission improves cognitive, but not motor deficits in a mouse model of Ataxia of Charlevoix-SaguenaySemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.