PXD043437
PXD043437 is an original dataset announced via ProteomeXchange.
Dataset Summary
| Title | The X-Linked Intellectual Disability gene, ZDHHC9, is important for oligodendrocyte maturation and myelin formation |
| Description | Two percent of all patients with X-linked intellectual disability (XLID) exhibit loss-of-function mutations in the palmitoylating enzyme, ZDHHC91,2. One of the main anatomical deficits observed in these patients is a decrease in corpus callosum volume and a disruption of white matter integrity. We demonstrated that ablation of Zdhhc9 in mice substantially impairs the maturation of ligodendrocytes, resulting in fewer mature, myelinating oligodendrocytes, higher numbers of oligodendrocyte progenitor cells and a decrease in the density of myelinated axons. Ultrastructural analysis of the remaining myelinated axons in the corpus callosum revealed further disruptions in myelin integrity. RNA sequencing and proteomic analyses revealed a concomitant decrease in the expression of genes and proteins involved in lipid metabolism, cholesterol synthesis and myelin compaction. These results reveal a previously underappreciated and fundamental role for ZDHHC9 and protein palmitoylation in regulating oligodendrocyte differentiation and myelinogenesis and provide mechanistic insights into the deficits observed in white matter volume in patients with mutations in ZDHHC9. |
| HostingRepository | MassIVE |
| AnnounceDate | 2023-08-22 |
| AnnouncementXML | Submission_2023-08-22_09:55:53.071.xml |
| DigitalObjectIdentifier | |
| ReviewLevel | Non peer-reviewed dataset |
| DatasetOrigin | Original dataset |
| RepositorySupport | Unsupported dataset by repository |
| PrimarySubmitter | Jason Rogalski |
| SpeciesList | scientific name: Mus musculus; common name: house mouse; NCBI TaxID: 10090; |
| ModificationList | Palmitoyl |
| Instrument | timsTOF Pro 2 |
Dataset History
| Revision | Datetime | Status | ChangeLog Entry |
|---|---|---|---|
| 0 | 2023-06-30 09:57:19 | ID requested | |
| ⏵ 1 | 2023-08-22 09:55:53 | announced |
Publication List
| no publication |
Keyword List
| submitter keyword: palmitoylation, myelin, X-linked intellectual disability |
Contact List
| Shernaz Xerxes Bamji | |
|---|---|
| contact affiliation | University of British Columbia |
| contact email | shernaz.bamji@ubc.ca |
| lab head | |
| Jason Rogalski | |
| contact affiliation | UBC |
| contact email | rogy@mail.ubc.ca |
| dataset submitter | |
Full Dataset Link List
| MassIVE dataset URI |
| 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://massive.ucsd.edu/MSV000092318/ |
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