scholarly journals Murine MPDZ-Linked Hydrocephalus is Caused by Hyperpermeability of the Choroid Plexus

2018 ◽  
Author(s):  
Junning Yang ◽  
Claire Simonneau ◽  
Robert Kilker ◽  
Laura Oakley ◽  
Matthew Byrne ◽  
...  
Keyword(s):  
Choroid Plexus ◽  
Protein Concentration ◽  
Cell Monolayer ◽  
Fold Increase ◽  
Loss Of Function ◽  
Choroid Plexus Epithelial Cell ◽  
Ultrastructural Evidence ◽  
The Brain ◽  
Severe Hydrocephalus ◽  
Choroid Plexus Epithelial

ABSTRACTThough 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 straight-forward and concise explanation for the pathophysiology of Mpdz-linked hydrocephalus.

scholarly journals Expression of junctional proteins in choroid plexus epithelial cell lines: a comparative study

2007 ◽  
Vol 4 (1) ◽  
pp. 11 ◽  
Author(s):  
Joanna Szmydynger-Chodobska ◽  
Crissey L Pascale ◽  
Andrew N Pfeffer ◽  
Cassaundra Coulter ◽  
Adam Chodobski
Keyword(s):  
Epithelial Cell ◽  
Comparative Study ◽  
Choroid Plexus ◽  
Cell Lines ◽  
Choroid Plexus Epithelial Cell ◽  
Junctional Proteins ◽  
Epithelial Cell Lines ◽  
Choroid Plexus Epithelial

scholarly journals TRIP6 functions in brain ciliogenesis

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Shalmali Shukla ◽  
Ronny Haenold ◽  
Pavel Urbánek ◽  
Lucien Frappart ◽  
Shamci Monajembashi ◽  
...  
Keyword(s):  
Choroid Plexus ◽  
Super Resolution ◽  
Epithelial Cell Line ◽  
Lim Domain ◽  
Choroid Plexus Epithelial Cell ◽  
Interaction Sites ◽  
Assembly Factor ◽  
Pericentriolar Material ◽  
Choroid Plexus Epithelial

AbstractTRIP6, a member of the ZYXIN-family of LIM domain proteins, is a focal adhesion component. Trip6 deletion in the mouse, reported here, reveals a function in the brain: ependymal and choroid plexus epithelial cells are carrying, unexpectedly, fewer and shorter cilia, are poorly differentiated, and the mice develop hydrocephalus. TRIP6 carries numerous protein interaction domains and its functions require homodimerization. Indeed, TRIP6 disruption in vitro (in a choroid plexus epithelial cell line), via RNAi or inhibition of its homodimerization, confirms its function in ciliogenesis. Using super-resolution microscopy, we demonstrate TRIP6 localization at the pericentriolar material and along the ciliary axoneme. The requirement for homodimerization which doubles its interaction sites, its punctate localization along the axoneme, and its co-localization with other cilia components suggest a scaffold/co-transporter function for TRIP6 in cilia. Thus, this work uncovers an essential role of a LIM-domain protein assembly factor in mammalian ciliogenesis.

scholarly journals Synthesis of transthyretin (pre-albumin) mRNA in choroid plexus epithelial cells, localized by in situ hybridization in rat brain.

1986 ◽  
Vol 34 (7) ◽  
pp. 949-952 ◽  
Author(s):  
A J Stauder ◽  
P W Dickson ◽  
A R Aldred ◽  
G Schreiber ◽  
F A Mendelsohn ◽  
...  
Keyword(s):  
In Situ Hybridization ◽  
Epithelial Cells ◽  
Rat Brain ◽  
Choroid Plexus ◽  
Cellular Localization ◽  
Lateral Ventricles ◽  
The Brain ◽  
Albumin Mrna ◽  
Choroid Plexus Epithelial

The sites of synthesis of transthyretin in the brain were investigated using in situ hybridization with [35S]-labeled recombinant cDNA probes specific for transthyretin mRNA. Autoradiography of hybridized coronal sections of rat brain revealed specific cellular localization of transthyretin mRNA in choroid plexus epithelial cells of the lateral, third, and fourth ventricles. Transferrin mRNA was also investigated and, in contrast to transthyretin mRNA, was localized mainly in the lateral ventricles. Our results indicate that substantial synthesis of transthyretin and transferrin mRNA may occur in the choroid plexus.

scholarly journals TNF Induces Choroid Plexus Epithelial Cell Barrier Alterations by Apoptotic and Nonapoptotic Mechanisms

2010 ◽  
Vol 2010 ◽  
pp. 1-10 ◽  
Author(s):  
Christian Schwerk ◽  
Kasia Rybarczyk ◽  
Frank Essmann ◽  
Annette Seibt ◽  
Marie-Louise Mölleken ◽  
...  
Keyword(s):  
Choroid Plexus ◽  
Inflammatory Diseases ◽  
High Mobility ◽  
Structural Basis ◽  
Choroid Plexus Epithelial Cell ◽  
The Central Nervous System ◽  
Cell Condensation ◽  
Dose Dependent Decrease ◽  
Dose Dependent ◽  
Choroid Plexus Epithelial

The choroid plexus epithelium constitutes the structural basis of the blood-cerebrospinal fluid barrier. Since the cytokine TNF is markedly increased during inflammatory diseases in the blood and the central nervous system, we investigated by which mechanisms TNF induces barrier alteration in porcine choroid plexus epithelial cells. We found a dose-dependent decrease of transepithelial electrical resistance, increase of paracellular inulin-flux, and induction of histone-associated DNA fragmentation and caspase-3 activation after TNF stimulation. This response was strongly aggravated by the addition of cycloheximide and could partially be inhibited by the NF-B inhibitor CAPE, but most effectively by the pan-caspase-inhibitor zVAD-fmk and not by the JNK inhibitor SP600125. Partial loss of cell viability could also be attenuated by CAPE. Immunostaining showed cell condensation and nuclear binding of high-mobility group box 1 protein as a sign of apoptosis after TNF stimulation. Taken together our findings indicate that TNF compromises PCPEC barrier function by caspase and NF-B dependent mechanisms.

scholarly journals Kinetic Profile of the Transcriptome Changes Induced in the Choroid Plexus by Peripheral Inflammation

2009 ◽  
Vol 29 (5) ◽  
pp. 921-932 ◽  
Author(s):  
Fernanda Marques ◽  
João C Sousa ◽  
Giovanni Coppola ◽  
Ana M Falcao ◽  
Ana João Rodrigues ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Choroid Plexus ◽  
Acute Phase ◽  
Acute Phase Proteins ◽  
Brain Parenchyma ◽  
Matrix Remodeling ◽  
Choroid Plexus Epithelial Cell ◽  
Blood Concentrations ◽  
The Brain

The choroid plexus, being part of the blood-brain barriers and responsible for the production of cerebrospinal fluid, is ideally positioned to transmit signals into and out of the brain. This study, using microarray analysis, shows that the mouse choroid plexus displays an acute-phase response after an inflammatory stimulus induced in the periphery by lipopolysaccharide (LPS). Remarkably, the response is specific to a restricted number of genes (out of a total of 24,000 genes analyzed, 252 are up-regulated and 173 are down-regulated) and transient, as it returns to basal conditions within 72 h. The up-regulated genes cluster into families implicated in immune-mediated cascades and in extracellular matrix remodeling, whereas those down-regulated participate in maintenance of the barrier function. Importantly, several acute-phase proteins, whose blood concentrations rise in response to inflammation, may contribute to the effects observed in vivo after LPS injection, as suggested by the differential response of primary choroid plexus epithelial cell cultures to LPS alone or to serum collected from animals exposed to LPS. By modulating the composition of the cerebrospinal fluid, which will ultimately influence the brain parenchyma, the choroid plexus response to inflammation may be of relevance in brain homeostasis in health and disease.

scholarly journals Fgf2 is expressed in human and murine embryonic choroid plexus and affects choroid plexus epithelial cell behaviour

2008 ◽  
Vol 5 (1) ◽  
pp. 20 ◽  
Author(s):  
Sarah Greenwood ◽  
Adam Swetloff ◽  
Angela M Wade ◽  
Tetsuya Terasaki ◽  
Patrizia Ferretti
Keyword(s):  
Epithelial Cell ◽  
Choroid Plexus ◽  
Choroid Plexus Epithelial Cell ◽  
Cell Behaviour ◽  
Choroid Plexus Epithelial
Sign in / Sign up

Export Citation Format

Share Document