Embryonic dentate fascia was grafted into the somatosensory neocortex of adult rats. Nine months post-grafting, the ultrastructural and morphometric analysis of the giant synapses established between the grafted granular neurons and inappropriate targets in the recipient brain was performed. As compared to the intact synaptic endings in the control hippocampus, differences were found in both the number and distribution of large dense-core synaptic vesicles, which store the neuropeptide co-transmitters. The peptidergic vesicle proportion (of total vesicle pool) within the ectopic giant synapses was 5.8 +/- 0.6% (versus 3.3 +/- 0.6% in the control). Clusters of large dense-core vesicles near the active zones of aberrant connections were observed almost 7.9 times more frequently than that of normal contacts. These data provide evidence that neuropeptide transmitters are critical for the maintenance of synaptic connections between the heterotopic dentate grafts and host brain.
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Seizure
December 2021
Clinic of Pediatric Neurology, Institute of Mother and Child, Warsaw, Poland.
Developmental and epileptic encephalopathies (DEE) constitute an expanding group of severely disabling and, most frequently, drug-resistant disorders starting in the first year of life. Among them, there is DEE43, caused by dominant mutations in the GABRB3 gene. We present first neuropathological findings in a novel, molecularly confirmed case with the fatal course.
View Article and Find Full Text PDFInt J Colorectal Dis
October 2019
Langone Health, Brooklyn, Brooklyn, NY, 11220, USA.
Background: Osseous metaplasia of the gastrointestinal tract is exceedingly rare. Associated with colorectal cancer, juvenile polyps , and inflammatory polyps, the exact etiology is still unknown. We present a case report on a young male with recurrent rectal polyps and rectal bleeding.
View Article and Find Full Text PDFDevelopment
June 2017
Université Côte d'Azur, CNRS, Inserm, iBV, Nice 06100, France
Development of the dentate gyrus (DG), the primary gateway for hippocampal inputs, spans embryonic and postnatal stages, and involves complex morphogenetic events. We have previously identified the nuclear receptor COUP-TFI as a novel transcriptional regulator in the postnatal organization and function of the hippocampus. Here, we dissect its role in DG morphogenesis by inactivating it in either granule cell progenitors or granule neurons.
View Article and Find Full Text PDFJ Med Genet
December 2016
Rare Disease Unit, Istituto Giannina Gaslini, Genoa, Italy.
Background: Fibrodysplasia ossificans progressiva is an autosomal dominant disorder due to germline mutations of ACVR1/ALK2 causing progressive heterotopic endochondral ossifications. Evidence of central nervous system involvement has emerged only recently.
Methods: We performed an observational cross-sectional brain MRI study in 13 patients (8 females, mean age 20 years), examining the relationship of clinical and neuroradiological findings.
Neuroscience
October 2015
Laboratory of Cerebrovascular Mechanisms of Brain Disorders, Department of Neuroscience, Institute of Functional Genomics, Montpellier (CNRS-INSERM), France. Electronic address:
Introduction: Neuro-vascular rearrangement occurs in brain disorders, including epilepsy. Platelet-derived growth factor receptor beta (PDGFRβ) is used as a marker of perivascular pericytes. Whether PDGFRβ(+) cell reorganization occurs in regions of neuro-vascular dysplasia associated with seizures is unknown.
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