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PXD041095

PXD041095 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleGranulins rescue inflammation, lysosome dysfunction, and neuropathology in a mouse model of progranulin deficiency
DescriptionIn this study, we investigate whether individual granulin subunits derived from the precursor protein progranulin are beneficial. We test this by asking if delivery of a single granulin to the brains of a preclinical model of FTD-GRN Grn-/-mice is sufficient to ameliorate disease-like phenotypes. We performed intracerebroventricular injections of recombinant adeno-associated virus (rAAV2/1) encoding granulin-2, granulin-4, full-length PGRN or GFP in neonatal Grn+/+ and Grn-/- mice. Following AAV injections, mice were aged for 12 months, tissues collected, and analyzed. Quantitative proteomics of the thalamus identified lysosomal function and neuroinflammation as pathways ameliorated by the expression of granulins. Biochemical markers of lysosomal dysfunction, neuroinflammation, and disease-like pathology were assessed via immunoblot and immunohistochemistry. We found that both granulin-2 and granulin-4 rescue markers of lysosomal dysfunction and neuroinflammation in cortical, hippocampal, and thalamic tissue.
HostingRepositoryPRIDE
AnnounceDate2024-10-22
AnnouncementXMLSubmission_2024-10-22_07:01:58.996.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterDuc Duong
SpeciesList scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationListphosphorylated residue; iodoacetamide derivatized residue
InstrumentOrbitrap Fusion Lumos
Dataset History
RevisionDatetimeStatusChangeLog Entry
02023-03-24 05:21:00ID requested
12024-10-14 17:28:22announced
22024-10-22 07:01:59announced2024-10-22: Updated project metadata.
Publication List
Keyword List
submitter keyword: Lipofuscin, CD68, Neurodegeneration, Inflammation,Progranulin (PGRN), BMP, Adeno-associated virus, Frontotemporal dementia (FTD), Lysosome, Galectin-3, Lipidomics, Granulins, Proteomics
Contact List
Thomas Kukar
contact affiliationPharmocology Asc Professor Emory University
contact emailtkukar@emory.edu
lab head
Duc Duong
contact affiliationCenter of Neurodegenerative Diseases
contact emaildduong@emory.edu
dataset submitter
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Dataset FTP location
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