scholarly journals ZFP423 regulates early patterning and multiciliogenesis in the hindbrain choroid plexus

2020 ◽  
Author(s):  
Filippo Casoni ◽  
Laura Croci ◽  
Francesca Vincenti ◽  
Paola Podini ◽  
Luca Massimino ◽  
...  
Keyword(s):  
Choroid Plexus ◽  
Cell Fate ◽  
Third Ventricle ◽  
Joubert Syndrome ◽  
Ependymal Cells ◽  
Cerebral Aqueduct ◽  
Cell Fate Specification ◽  
Stem Cell Maintenance ◽  
Roof Plate ◽  
Secretory Tissue

ABSTRACTThe choroid plexus (ChP) is a secretory tissue that produces cerebrospinal fluid (CSF) and secretes it into the ventricular system. CSF flows from the lateral to the third ventricle, and then to the fourth ventricle through the cerebral aqueduct. Recent studies have uncovered new, active roles for this structure in the regulation of neural stem cell maintenance and differentiation into neurons. Zfp423, encoding a Kruppel-type zinc finger transcription factor essential for cerebellar development and mutated in rare cases of cerebellar vermis hypoplasia / Joubert syndrome and other ciliopathies, is expressed in the hindbrain roof plate (RP), from which the IV ventricle ChP arises, and in mesenchymal cells giving rise to the stroma and leptomeninges. Zfp423 mutants display a marked reduction of the hindbrain ChP (hChP), which fails to express key markers of its secretory function and genes implicated in its development and maintenance (Lmx1a, Otx2). The mutant hChP displays a complete lack of multiciliated ependymal cells. A transcriptome analysis conducted at the earliest stages of hChP development and subsequent validations demonstrate that the mutant hChp displays a strong deregulation of pathways involved in early hindbrain patterning and multiciliated cell fate specification. Our results propose Zfp423 as a master gene and one of the earliest known determinants of hChP development.

scholarly journals ZFP423 regulates early patterning and multiciliogenesis in the hindbrain choroid plexus

Development ◽  
2020 ◽  
Vol 147 (22) ◽  
pp. dev190173
Author(s):  
Filippo Casoni ◽  
Laura Croci ◽  
Francesca Vincenti ◽  
Paola Podini ◽  
Michela Riba ◽  
...  
Keyword(s):  
Epithelial Cells ◽  
Choroid Plexus ◽  
Joubert Syndrome ◽  
Mesenchymal Cells ◽  
Secretory Function ◽  
Zinc Finger Transcription Factor ◽  
Master Genes ◽  
Roof Plate ◽  
Mesodermal Origin ◽  
Secretory Tissue

ABSTRACTThe choroid plexus (ChP) is a secretory tissue that produces cerebrospinal fluid (CSF) secreted into the ventricular system. It is a monolayer of secretory, multiciliated epithelial cells derived from neuroepithelial progenitors and overlying a stroma of mesenchymal cells of mesodermal origin. Zfp423, which encodes a Kruppel-type zinc-finger transcription factor essential for cerebellar development and mutated in rare cases of cerebellar vermis hypoplasia/Joubert syndrome and other ciliopathies, is expressed in the hindbrain roof plate, from which the IV ventricle ChP arises, and, later, in mesenchymal cells, which give rise to the stroma and leptomeninges. Mouse Zfp423 mutants display a marked reduction of the hindbrain ChP (hChP), which: (1) fails to express established markers of its secretory function and genes implicated in its development and maintenance (Lmx1a and Otx2); (2) shows a perturbed expression of signaling pathways previously unexplored in hChP patterning (Wnt3); and (3) displays a lack of multiciliated epithelial cells and a profound dysregulation of master genes of multiciliogenesis (Gmnc). Our results propose that Zfp423 is a master gene and one of the earliest known determinants of hChP development.

Migrating Lateral Ventricle Choroid Plexus Cyst into the Fourth Ventricle Causing Acute Hydrocephalus

2021 ◽  
Author(s):  
Lacey M. Carter ◽  
Benjamin Cornwell ◽  
Naina L. Gross
Keyword(s):  
Cerebrospinal Fluid ◽  
Choroid Plexus ◽  
Lateral Ventricle ◽  
Fourth Ventricle ◽  
Third Ventricle ◽  
Neurological Deficits ◽  
Cerebral Aqueduct ◽  
Outflow Obstruction ◽  
The Third ◽  
Choroid Plexus Cyst

AbstractChoroid plexus cysts consist of abnormal folds of the choroid plexus that typically resolve prior to birth. Rarely, these cysts persist and may cause outflow obstruction of cerebrospinal fluid. We present a 5-month-old male born term who presented with lethargy, vomiting, and a bulging anterior fontanelle. Magnetic resonance imaging showed one large choroid plexus cyst had migrated from the right lateral ventricle through the third ventricle and cerebral aqueduct into the fourth ventricle causing outflow obstruction. The cyst was attached to the lateral ventricle choroid plexus by a pedicle. The cyst was endoscopically retrieved from the fourth ventricle intact and then fenestrated and coagulated along with several other smaller cysts. Histologic examination confirmed the mass was a choroid plexus cyst. The patient did well after surgery and did not require any cerebrospinal fluid diversion. Nine months after surgery, the patient continued to thrive with no neurological deficits. This case is the first we have found in the literature of a lateral ventricular choroid plexus cyst migrating into the fourth ventricle and the youngest of any migrating choroid plexus cyst. Only three other cases of a migrating choroid plexus cyst have been documented and those only migrated into the third ventricle. New imaging advances are making these cysts easier to identify, but may still be missed on routine sequences. High clinical suspicion for these cysts is necessary for correct treatment of this possible cause of hydrocephalus.

Intracranial Pressure

2019 ◽  
pp. 69-73
Author(s):  
Eelco F. M. Wijdicks ◽  
William D. Freeman
Keyword(s):  
Cerebrospinal Fluid ◽  
Smooth Muscle ◽  
Choroid Plexus ◽  
Blood Cells ◽  
White Blood Cells ◽  
Ependymal Cells ◽  
Cerebral Aqueduct ◽  
Epithelial Surface ◽  
The Brain ◽  
Route Of Delivery

Cerebrospinal fluid (CSF) fills the subarachnoid space, spinal canal, and ventricles of the brain. CSF is enclosed within the brain by the pial layer, ependymal cells lining the ventricles, and the epithelial surface of the choroid plexus, where it is largely produced. Choroid plexus is present throughout the ventricular system with the exception of the frontal and occipital horns of the lateral ventricle and the cerebral aqueduct. The vascular smooth muscle and the epithelium of the choroid plexus receive both sympathetic and parasympathetic input. In an adult, CSF is normally acellular. A normal spinal sample may contain up to 5 white blood cells (WBCs) or red blood cells (RBCs). CSF allows for a route of delivery and removal of nutrients, hormones, and transmitters for the brain.

scholarly journals The Lactate Receptor HCA1 Is Present in the Choroid Plexus, the Tela Choroidea, and the Neuroepithelial Lining of the Dorsal Part of the Third Ventricle

2020 ◽  
Vol 21 (18) ◽  
pp. 6457 ◽  
Author(s):  
Alena Hadzic ◽  
Teresa D. Nguyen ◽  
Makoto Hosoyamada ◽  
Naoko H. Tomioka ◽  
Linda H. Bergersen ◽  
...  
Keyword(s):  
Choroid Plexus ◽  
Fluorescent Protein ◽  
Third Ventricle ◽  
Ependymal Cells ◽  
Dorsal Part ◽  
Brain Physiology ◽  
The Third ◽  
Tela Choroidea ◽  
Csf Lactate ◽  
Lactate Levels

The volume, composition, and movement of the cerebrospinal fluid (CSF) are important for brain physiology, pathology, and diagnostics. Nevertheless, few studies have focused on the main structure that produces CSF, the choroid plexus (CP). Due to the presence of monocarboxylate transporters (MCTs) in the CP, changes in blood and brain lactate levels are reflected in the CSF. A lactate receptor, the hydroxycarboxylic acid receptor 1 (HCA1), is present in the brain, but whether it is located in the CP or in other periventricular structures has not been studied. Here, we investigated the distribution of HCA1 in the cerebral ventricular system using monomeric red fluorescent protein (mRFP)-HCA1 reporter mice. The reporter signal was only detected in the dorsal part of the third ventricle, where strong mRFP-HCA1 labeling was present in cells of the CP, the tela choroidea, and the neuroepithelial ventricular lining. Co-labeling experiments identified these cells as fibroblasts (in the CP, the tela choroidea, and the ventricle lining) and ependymal cells (in the tela choroidea and the ventricle lining). Our data suggest that the HCA1-containing fibroblasts and ependymal cells have the ability to respond to alterations in CSF lactate in body–brain signaling, but also as a sign of neuropathology (e.g., stroke and Alzheimer’s disease biomarker).

scholarly journals A Novel Model of Acquired Hydrocephalus for Evaluation of Neurosurgical Treatments

2021 ◽  
Author(s):  
James (Pat) Mcallister ◽  
Michael Talcott ◽  
Albert M. Isaacs ◽  
Leandro Castaneyra-Ruiz ◽  
Sarah H. Zwick ◽  
...  
Keyword(s):  
Ventricular Zone ◽  
Autologous Blood ◽  
Third Ventricle ◽  
Recovery Period ◽  
Neurological Status ◽  
Ependymal Cells ◽  
Cerebral Aqueduct ◽  
Ventricular Catheter ◽  
Post Induction

Abstract Background.Many animal models have been used to study the pathophysiology of hydrocephalus; most of these have been rodent models whose lissencephalic cerebral cortex may not respond to ventriculomegaly in ways similar to gyrencephalic species and whose size is not amenable to evaluation of clinically-relevant neurosurgical treatments. Fewer models of hydrocephalus in gyrencephalic species have been used; thus, we have expanded upon a porcine model of hydrocephalus in juvenile pigs. Methods. Acquired hydrocephalus was induced in 30-35-day old pigs by percutaneous intracisternal injections of kaolin. Intracisternal and intraventricular injections of autologous blood was attempted in 2 cases to induce post-hemorrhagic hydrocephalus. Magnetic resonance imaging (MRI) was employed to evaluate the progression of ventriculomegaly and plan the surgical implantation of ventriculoperitoneal shunts at approximately 1–4 weeks post-kaolin. Behavioral and neurological status was assessed continuously. Results. Bilateral ventriculomegaly occurred post-induction and was characterized by enlargement of all portions of the cerebral ventricles, with prominent CSF flow voids in the third ventricle, foramina of Monro, and cerebral aqueduct. Kaolin deposits formed a solid cast in basal cisterns but the cisterna magna was patent. In 14 untreated hydrocephalic animals, mean total ventricular volumes were 6786 ± 4336 SD mm3 at 17–57 days post-kaolin, which was significantly larger than the baseline values of 2251 ± 194 SD mm3 in sham controls. Consistent with human hydrocephalus, intermittent disruption of the ventricular zone of the lateral ventricles was characterized by loss of multiciliated ependymal cells and the appearance of reactive astrocytes. Past the post-induction recovery period, untreated pigs were asymptomatic in spite of exhibiting mild-moderate ventriculomegaly. Shunted animals developed ataxia and lethargy only when obstruction of the ventricular catheter and/or distal valve occurred. Conclusions. Mechanical induction of acquired hydrocephalus produces a reliable in vivo model that is highly translational, allowing systematic studies of the pathophysiology and clinical treatment of hydrocephalus.

Rat placental lactogen-I binds to the choroid plexus and hypothalamus of the pregnant rat

1993 ◽  
Vol 139 (2) ◽  
pp. 235-NP ◽  
Author(s):  
C. Pihoker ◽  
M. C. Robertson ◽  
M. Freemark
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Rat Brain ◽  
Choroid Plexus ◽  
Binding Sites ◽  
Third Ventricle ◽  
Placental Lactogen ◽  
Ependymal Cells ◽  
Pregnant Rat ◽  
The Third

ABSTRACT Recent findings suggest that placental lactogen has a role in the regulation of hypothalamic function during pregnancy. To explore the mechanisms by which placental hormones may exert effects in the maternal central nervous system, we have examined the binding of rat placental lactogen-I (rPL-I) to brain slices from pregnant rats at mid- and late gestation. The binding of rPL-I to maternal rat brain was compared with that of human GH (hGH). Radiolabelled rPL-I bound specifically to ependymal cells of the choroid plexus in the lateral ventricles and in the roof of the third ventricle. The binding of 125I-labelled rPL-I was inhibited by unlabelled rPL-I, hGH or rat prolactin but not by rat GH, indicating that rPL-I and rat prolactin interact with a common binding site in maternal rat brain. Radiolabelled hGH bound to the choroid plexus and to ependymal cells lining the third ventricle in the region of the arcuate nucleus. In addition, hGH bound specifically to the ventromedial nuclei and to the medial preoptic area of the hypothalamus. The binding of radiolabelled hGH to all brain regions was inhibited by unlabelled rPL-I as well as hGH, indicating that rPL-I competes for lactogenic binding sites in the hypothalamus as well as the choroid plexus of the pregnant rat. These findings suggest potential mechanisms by which placental hormones may exert direct effects on the maternal central nervous system during pregnancy. The precise functions and roles of the PL-I binding sites in maternal choroid plexus and hypothalamus remain to be explored. Journal of Endocrinology (1993) 139, 235–242

Development and evaluation of a patient-specific surgical simulator for endoscopic colloid cyst resection

2020 ◽  
Vol 133 (2) ◽  
pp. 521-529 ◽  
Author(s):  
Vivek P. Bodani ◽  
Gerben E. Breimer ◽  
Faizal A. Haji ◽  
Thomas Looi ◽  
James M. Drake
Keyword(s):  
Choroid Plexus ◽  
Computer Aided Design ◽  
Third Ventricle ◽  
Colloid Cyst ◽  
Comfort Level ◽  
Complex Case ◽  
Patient Specific ◽  
Surgical Simulator ◽  
Perceived Realism ◽  
Noncontrast Ct

OBJECTIVEEndoscopic resection of third-ventricle colloid cysts is technically challenging due to the limited dexterity and visualization provided by neuroendoscopic instruments. Extensive training and experience are required to master the learning curve. To improve the education of neurosurgical trainees in this procedure, a synthetic surgical simulator was developed and its realism, procedural content, and utility as a training instrument were evaluated.METHODSThe simulator was developed based on the neuroimaging (axial noncontrast CT and T1-weighted gadolinium-enhanced MRI) of an 8-year-old patient with a colloid cyst and hydrocephalus. Image segmentation, computer-aided design, rapid prototyping (3D printing), and silicone molding techniques were used to produce models of the skull, brain, ventricles, and colloid cyst. The cyst was filled with a viscous fluid and secured to the roof of the third ventricle. The choroid plexus and intraventricular veins were also included. Twenty-four neurosurgical trainees performed a simulated colloid cyst resection using a 30° angled endoscope, neuroendoscopic instruments, and image guidance. Using a 19-item feedback survey (5-point Likert scales), participants evaluated the simulator across 5 domains: anatomy, instrument handling, procedural content, perceived realism, and confidence and comfort level.RESULTSParticipants found the simulator’s anatomy to be highly realistic (mean 4.34 ± 0.63 [SD]) and appreciated the use of actual instruments (mean 4.38 ± 0.58). The procedural content was also rated highly (mean 4.28 ± 0.77); however, the perceived realism was rated slightly lower (mean 4.08 ± 0.63). Participants reported greater confidence in their ability to perform an endoscopic colloid cyst resection after using the simulator (mean 4.45 ± 0.68). Twenty-three participants (95.8%) indicated that they would use the simulator for additional training. Recommendations were made to develop complex case scenarios for experienced trainees (normal-sized ventricles, choroid plexus adherent to cyst wall, bleeding scenarios) and incorporate advanced instrumentation such as side-cutting aspiration devices.CONCLUSIONSA patient-specific synthetic surgical simulator for training residents and fellows in endoscopic colloid cyst resection was successfully developed. The simulator’s anatomy, instrument handling, and procedural content were found to be realistic. The simulator may serve as a valuable educational tool to learn the critical steps of endoscopic colloid cyst resection, develop a detailed understanding of intraventricular anatomy, and gain proficiency with bimanual neuroendoscopic techniques.

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