Lipid peroxidation is known to be associated with many neurodegenerative diseases and with traumatic brain injury, but its occurrence in the normal developing brain has not been reported. The present study was carried out using a specific antibody that recognises proteins modified by the end-product of lipid peroxide decomposition, 4-hydroxynonenal (HNE), to evaluate evaluate possible lipid peroxidation products in the brains of developing rats by immunocytochemistry and electron microscopy. Moderately dense labelling was observed in the supraventricular corpus callosum in the 7- and 8-day-old rats, whilst very dense labelling was observed in the same region, in the 9- and 10-day-old rats. Very little immunoreactivity was observed at 14 days, and no staining was observed in the corpus callosum in adult rats. HNE staining was not observed in neuronal cell bodies that give rise to callosal axons in the overlying cerebral cortex. Electron microscopy showed dense HNE staining on the basal laminae of blood vessels and on the plasma membranes of unmyelinated axons. Large numbers of rounded cells with features of oligodendrocyte precursor cells were labelled by Perl's stain in the supraventricular corpus callosum at postnatal day 7 and postnatal day 10, i.e. at times corresponding to high levels of HNE immunoreactivity. In contrast, very few such cells were observed in the adult brain, corresponding to the very little or no Perl's staining in the adult. These results suggest that lipid peroxidation observed in the supraventricular corpus callosum at postnatal day 10 could result from an accumulation of iron in this region, at this time.
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http://dx.doi.org/10.1007/s002210000625 | DOI Listing |
Eur J Pediatr
December 2024
Department of Medical Genetics, Dr. Behçet Uz Children's Hospital, Izmir, Turkey.
Unlabelled: The RASopathies are a group of disorders resulting from a germline variant in the genes encoding the Ras/mitogen-activated protein kinase pathway. These disorders include Noonan syndrome (NS), cardiofaciocutaneous syndrome (CFC), Costello syndrome (CS), Legius syndrome (LS), and neurofibromatosis type 1 (NF1), and have overlapping clinical features due to RAS/MAPK dysfunction. In this study, we aimed to describe the clinical and molecular features of patients exhibiting phenotypic manifestations consistent with RASopathies.
View Article and Find Full Text PDFACS Chem Neurosci
December 2024
Department of Radiology, The Second Affiliated Hospital, Zhejiang University of Medicine, Hangzhou 310009, China.
Clin Radiol
November 2024
Department of Radiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Aim: This study aimed to summarise and analyse the magnetic resonance imaging (MRI) characteristics of patients with myelin oligodendrocyte glycoprotein-immunoglobulin G-associated disease (MOGAD), and to enhance the accuracy of disease diagnosis and advance scientific research.
Materials And Methods: A retrospective collection of clinical data from 103 patients with MOGAD was conducted. The distribution and signal characteristics of intracranial lesions on MRI were analysed.
Front Immunol
December 2024
Department of Radiology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China.
Objective: Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a novel steroid sensitive autoimmune disease, without a diagnostic consensus. The purpose of this study was to improve early GFAP-A diagnosis by increasing awareness of key clinical characteristics and imaging manifestations.
Methods: Medical records of 13 patients with anti-GFAP antibodies in serum or cerebrospinal fluid (CSF) were reviewed for cross-sectional and longitudinal analysis of clinical and magnetic resonance imaging (MRI) findings.
Neurol Genet
February 2025
Department of Neuroscience, Mayo Clinic, Jacksonville, FL.
Objectives: In this study, we describe a 54-year-old Indian woman who presented with clinical features of Kufs syndrome A (KSA) and Kufs syndrome B (KSB), as well as neuropathologic and genetic findings consistent with neuronal ceroid lipofuscinosis type 13 (CLN13). Subsequently, we review the clinicopathologic features of 20 patients with CLN13 reported in the literature.
Methods: Data and imaging were obtained from the patient's medical records.
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