PXD050916 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | Restoring retinal polyunsaturated fatty acid balance and retina function by targeting ceramide in Adiponectin receptor 1 (AdipoR1) deficient mice |
Description | Mutations in the adiponectin receptor 1 gene (AdipoR1) lead to retinitis pigmentosa and are associated with age-related macular degeneration (AMD). This study explores the effects of AdipoR1 gene deficiency in mice, revealing a striking decline in ω3 polyunsaturated fatty acids (PUFA), an increase in ω6 FAs, and elevated ceramides in the retina. The AdipoR1 deficiency impairs peroxisome proliferator-activated receptor α (PPARα) signaling, which is crucial for FA metabolism, particularly affecting proteins associated with FA transport and oxidation in the retina and retinal pigmented epithelium (RPE). Our lipidomic and proteomic analyses indicate changes that could affect membrane composition and viscosity through altered ω3 PUFA transport and synthesis, suggesting a potential influence of AdipoR1 on these properties. Furthermore, we noted a reduction in the Bardet-Biedl syndrome (BBS) proteins, which are crucial for forming and maintaining photoreceptor outer segments that are PUFA-enriched ciliary structures. Diminution in BBS-proteins content combined with our electron microscopic observations raises the possibility that AdipoR1 deficiency might impair ciliary function. Treatment with inhibitors of ceramide synthesis led to substantial elevation of ω3 LC-PUFAs, alleviating photoreceptor degeneration and improving retinal function. These results serve as the proof of concept for a ceramide-targeted strategy to treat retinopathies linked to PUFA deficiency, including AMD. |
HostingRepository | PRIDE |
AnnounceDate | 2024-10-22 |
AnnouncementXML | Submission_2024-10-22_06:46:57.382.xml |
DigitalObjectIdentifier | |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Unsupported dataset by repository |
PrimarySubmitter | Fangyuan Gao |
SpeciesList | scientific name: Mus musculus (Mouse); NCBI TaxID: 10090; |
ModificationList | No PTMs are included in the dataset |
Instrument | Orbitrap Fusion Lumos |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2024-03-22 16:14:00 | ID requested | |
1 | 2024-06-23 01:39:33 | announced | |
⏵ 2 | 2024-10-22 06:46:59 | announced | 2024-10-22: Updated project metadata. |
Publication List
Lewandowski D, Gao F, Imanishi S, Tworak A, Bassetto M, Dong Z, Pinto AFM, Tabaka M, Kiser PD, Imanishi Y, Skowronska-Krawczyk D, Palczewski K, Restoring retinal polyunsaturated fatty acid balance and retina function by targeting ceramide in AdipoR1-deficient mice. J Biol Chem, 300(5):107291(2024) [pubmed] |
10.1016/j.jbc.2024.107291; |
Keyword List
submitter keyword: proteomics, lipidomics, retinal pigment epithelium (RPE), retina,Polyunsaturated fatty acids (PUFAs) |
Contact List
Dominik Lewandowski |
contact affiliation | Department of Ophthalmology, University of California, Irvine, CA 92697 |
contact email | lewandod@hs.uci.edu |
lab head | |
Fangyuan Gao |
contact affiliation | University of California, Irvine |
contact email | fangyuag@hs.uci.edu |
dataset submitter | |
Full Dataset Link List
Dataset FTP location
NOTE: Most web browsers have now discontinued native support for FTP access within the browser window. But you can usually install another FTP app (we recommend FileZilla) and configure your browser to launch the external application when you click on this FTP link. Or otherwise, launch an app that supports FTP (like FileZilla) and use this address: ftp://ftp.pride.ebi.ac.uk/pride/data/archive/2024/06/PXD050916 |
PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD050916
- Label: PRIDE project
- Name: Restoring retinal polyunsaturated fatty acid balance and retina function by targeting ceramide in Adiponectin receptor 1 (AdipoR1) deficient mice