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http://dx.doi.org/10.1136/jnnp-2015-310601 | DOI Listing |
Neurobiol Dis
January 2025
KU Leuven - University of Leuven, Department of Neurosciences and Leuven Brain Institute (LBI), Leuven, Belgium; Laboratory of Neurobiology, VIB Center for Brain & Disease Research, Leuven, Belgium. Electronic address:
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder characterized by the selective and progressive loss of motor neurons, leading to gradual paralysis and death within 2 to 5 years after diagnosis. The exact underlying pathogenic mechanism(s) remain elusive. This is particularly the case for sporadic ALS (sALS), representing 90 % of cases, as modelling a sporadic disease is extremely difficult.
View Article and Find Full Text PDFASN Neuro
January 2025
Department of Anesthesiology, University of Illinois at Chicago, Chicago, Illinois, USA.
Despite tremendous progress in characterizing the myriad cellular structures in the nervous system, a full appreciation of the interdependent and intricate interactions between these structures is as yet unfulfilled. Indeed, few more so than the interaction between the myelin internode and its ensheathed axon. More than a half-century after the ultrastructural characterization of this axomyelin unit, we lack a reliable understanding of the physiological properties, the significance and consequence of pathobiological processes, and the means to gauge success or failure of interventions designed to mitigate disease.
View Article and Find Full Text PDFBiol Open
January 2025
Seaver Autism Center for Research and Treatment, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA.
Cell fate decisions during cortical development sculpt the identity of long-range connections that subserve complex behaviors. These decisions are largely dictated by mutually exclusive transcription factors, including CTIP2/Bcl11b for subcerebral projection neurons and BRN1/Pou3f3 for intra-telencephalic projection neurons. We have recently reported that the balance of cortical CTIP2-expressing neurons is altered in a mouse model of DDX3X syndrome, a female-biased neurodevelopmental disorder associated with intellectual disability, autism spectrum disorder, and significant motor challenges.
View Article and Find Full Text PDFFront Immunol
January 2025
Neuroimmunology Research Group, Netherlands Institute for Neuroscience, Amsterdam, Netherlands.
Introduction: Remyelination of demyelinated axons can occur as an endogenous repair mechanism in multiple sclerosis (MS), but its efficacy varies between both MS individuals and lesions. The molecular and cellular mechanisms that drive remyelination remain poorly understood. Here, we studied the relation between microglia activation and remyelination activity in MS.
View Article and Find Full Text PDFSci Rep
January 2025
Department of Neurosurgery of the Second Affiliated Hospital, Interdisciplinary Institute of Neuroscience and Technology, School of Medicine, Zhejiang University, Hangzhou, China.
Horizontal connections in anterior inferior temporal cortex (ITC) are thought to play an important role in object recognition by integrating information across spatially separated functional columns, but their functional organization remains unclear. Using a combination of optical imaging, electrophysiological recording, and anatomical tracing, we investigated the relationship between stimulus-response maps and patterns of horizontal axon terminals in the macaque ITC. In contrast to the "like-to-like" connectivity observed in the early visual cortex, we found that horizontal axons in ITC do not preferentially connect sites with similar object selectivity.
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