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PXD050916

PXD050916 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleRestoring retinal polyunsaturated fatty acid balance and retina function by targeting ceramide in Adiponectin receptor 1 (AdipoR1) deficient mice
DescriptionMutations 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.
HostingRepositoryPRIDE
AnnounceDate2024-10-22
AnnouncementXMLSubmission_2024-10-22_06:46:57.382.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterFangyuan Gao
SpeciesList scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationListNo PTMs are included in the dataset
InstrumentOrbitrap Fusion Lumos
Dataset History
RevisionDatetimeStatusChangeLog Entry
02024-03-22 16:14:00ID requested
12024-06-23 01:39:33announced
22024-10-22 06:46:59announced2024-10-22: 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 affiliationDepartment of Ophthalmology, University of California, Irvine, CA 92697
contact emaillewandod@hs.uci.edu
lab head
Fangyuan Gao
contact affiliationUniversity of California, Irvine
contact emailfangyuag@hs.uci.edu
dataset submitter
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Dataset FTP location
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