PXD033823 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | Sacsin, the ARSACS disease protein, controls lysosomal positioning and reformation by regulating microtubule dynamics |
Description | Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a fatal brain disorder featuring cerebellar neurodegeneration leading to spasticity and ataxia. ARSACS is caused by mutations in the SACS gene that encodes sacsin, a massive 4579 amino acid protein with multiple modular domains. Here we demonstrate that sacsin binds to microtubules and regulates microtubule dynamics. Loss of sacsin function in knockout cell lines, knockdown and knockout neurons, and patient fibroblasts leads to alterations in lysosomal transport, positioning, function and reformation following autophagy. |
HostingRepository | PRIDE |
AnnounceDate | 2023-11-14 |
AnnouncementXML | Submission_2023-11-14_07:34:34.271.xml |
DigitalObjectIdentifier | |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Unsupported dataset by repository |
PrimarySubmitter | Vincent Francis |
SpeciesList | scientific name: Homo sapiens (Human); NCBI TaxID: 9606; |
ModificationList | monohydroxylated residue; iodoacetamide derivatized residue |
Instrument | Orbitrap Fusion |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2022-05-12 02:03:25 | ID requested | |
1 | 2022-10-14 22:51:59 | announced | |
⏵ 2 | 2023-11-14 07:34:35 | announced | 2023-11-14: Updated project metadata. |
Publication List
Francis V, Alshafie W, Kumar R, Girard M, Brais B, McPherson PS, The ARSACS disease protein sacsin controls lysosomal positioning and reformation by regulating microtubule dynamics. J Biol Chem, 298(9):102320(2022) [pubmed] |
Keyword List
submitter keyword: Lysosome, ALR, Microtubule |
Contact List
Peter McPherson |
contact affiliation | James McGill Professor, Montreal Neurological Institute, McGill University |
contact email | peter.mcpherson@mcgill.ca |
lab head | |
Vincent Francis |
contact affiliation | McGill University |
contact email | vincent.francis@mail.mcgill.ca |
dataset submitter | |
Full Dataset Link List
Dataset FTP location
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PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD033823
- Label: PRIDE project
- Name: Sacsin, the ARSACS disease protein, controls lysosomal positioning and reformation by regulating microtubule dynamics