PXD011535

PXD011535 is an original dataset announced via ProteomeXchange.

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

Revision	Datetime	Status	ChangeLog Entry
0	2018-10-30 09:37:20	ID requested
⏵ 1	2018-11-23 06:58:21	announced

Dataset with its publication pending

curator keyword: Biological

submitter keyword: Mpdz, hydrocephalus, choroid plexus, cerebrospinal fluid, mouse

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

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

• PRIDE
  1. PXD011535
     1. Label: PRIDE project
     2. Name: Composition of the cerebrospinal fluid of normal and Mpdz-linked hydrocephalus-harboring mice