PXD011535 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | Composition of the cerebrospinal fluid of normal and Mpdz-linked hydrocephalus-harboring mice |
Description | Though congenital hydrocephalus is heritable, it has been linked only to eight genes, one of which is MPDZ. Humans and mice that carry a truncated version of MPDZ incur severe hydrocephalus resulting in acute morbidity and lethality. We show by magnetic resonance imaging that contrast-medium penetrates into the brain ventricles of mice carrying a Mpdz loss-of-function mutation, whereas none is detected in the ventricles of normal mice, implying that the permeability of the choroid plexus epithelial cell monolayer is abnormally high. Comparative proteomic analysis of the cerebrospinal fluid of normal and hydrocephalic mice revealed up to a 53-fold increase in protein concentration, suggesting that transcytosis through the choroid plexus epithelial cells of Mpdz KO mice is substantially higher than in normal mice. These conclusions are supported by ultrastructural evidence, and by immunohistochemistry and cytology data. Our results provide a straightforward and concise explanation for the pathophysiology of Mpdz-linked hydrocephalus. |
HostingRepository | PRIDE |
AnnounceDate | 2018-11-23 |
AnnouncementXML | Submission_2018-11-23_06:58:20.xml |
DigitalObjectIdentifier | |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Unsupported dataset by repository |
PrimarySubmitter | Arie Horowitz |
SpeciesList | scientific name: Mus musculus (Mouse); NCBI TaxID: 10090; |
ModificationList | monohydroxylated residue; iodoacetamide derivatized residue |
Instrument | LTQ Orbitrap Elite |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2018-10-30 09:37:20 | ID requested | |
⏵ 1 | 2018-11-23 06:58:21 | announced | |
Publication List
Dataset with its publication pending |
Keyword List
curator keyword: Biological |
submitter keyword: Mpdz, hydrocephalus, choroid plexus, cerebrospinal fluid, mouse |
Contact List
Arie Horowitz |
contact affiliation | Cardeza Center for Vascular Biology Sidney Kimmel Medical College Thomas Jefferson University |
contact email | arie.horowitz@jefferson.edu |
lab head | |
Arie Horowitz |
contact affiliation | Thomas Jefferson University |
contact email | arie.horowitz@jefferson.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/2018/11/PXD011535 |
PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD011535
- Label: PRIDE project
- Name: Composition of the cerebrospinal fluid of normal and Mpdz-linked hydrocephalus-harboring mice