• Pathology of affected optic nerves exhibits inflammation, demyelination, gliosis, axonal injury, and thinning of the retinal nerve fiber layer (RNFL). (tjn.org.tr)
  • This suggests that, in most cases, axonal damage is the root cause of the neuropathy, not demyelination. (medscape.com)
  • On the other hand, axonal damage can result in secondary demyelination. (medscape.com)
  • Neurotoxins may damage neurons, axons, and/or glia resulting in loss of specific nuclei and/or axonal tracts or demyelination. (medscape.com)
  • Patients with primary Sjögren syndrome show loss of WM microstructural integrity, probably related to both Wallerian degeneration and demyelination. (ajnr.org)
  • Two basic forms of nervous system disorders discussed were axonal degeneration and segmental demyelination. (cdc.gov)
  • GBS with axonal degeneration may occur without demyelination or inflammation. (medscape.com)
  • In collaboration with the Brain and Mind Centre and the Department of Neurology at the Royal North Shore Hospital, we are studying the mechanisms of demyelination/remyelination and axonal loss in multiple sclerosis. (edu.au)
  • This project uses the visual system as a model to examine the effect of chronic demyelination on axonal neurodegeneration in multiple sclerosis. (edu.au)
  • Moreover, establishing microfluidic platforms that mimic the compartmentalized nature of RGC axonal degeneration in glaucoma will allow for the study of other mechanisms involved in glaucomatous neurodegeneration. (brightfocus.org)
  • This workshop aims to explore the mechanisms leading to neuronal dysfunction and degeneration, and address how neurons can react to injury by promoting a regenerative program, by reuniting leading researchers in the field of axonal degeneration/regeneration to paint an updated picture of the field and explore new discoveries and methodologies with the goal of deepening our understanding of these subjects. (oist.jp)
  • More broadly, we are interested in the molecular mechanisms underlying axonal degeneration, with a focus on the role of mRNA translation defects. (europeandrosophilasociety.org)
  • Through mechanisms that at present can only be speculated about, myelin disturbances result in axonal damage. (medscape.com)
  • Among the conclusions: In ALS models, reduction of organelle transport and initiation of axon degeneration appear to be due to different mechanisms. (mytum.de)
  • We generated a transgenic mouse model of CMT2A that developed severe early onset vision loss and neurological deficits, axonal degeneration without cell body loss, and cytoplasmic and axonal accumulations of fragmented mitochondria. (wustl.edu)
  • Their results show that transport deficits and axon degeneration can develop independently, refuting the hypothesis that one is a direct cause of the other. (mytum.de)
  • These mutants display adult-onset progressive degeneration of peripheral motor and/or sensory neurons. (academictransfer.com)
  • Pharmacological inhibition of the necroptotic kinase RIPK1 using necrostatin-1 strongly delayed axonal degeneration in the peripheral nervous system and CNS of wild-type mice of either sex and protected in vitro sensory axons from degeneration after mechanical and toxic insults. (lu.se)
  • In CNS and peripheral nervous system diseases associated with axonal injury or degeneration, the concentration of neurofilament light chain (NfL) has been found to increase in cerebrospinal fluid (CSF) and blood. (bmj.com)
  • Demyelinating or axonal peripheral neuropathies can occur. (msdmanuals.com)
  • Its principal toxic side effect is axonal degeneration resulting in peripheral neuropathy. (bvsalud.org)
  • DAI is characterized by axonal separation, in which the axon is torn at the site of stretch and the part distal to the tear degrades by a process known as Wallerian degeneration. (wikipedia.org)
  • When the axon is torn, Wallerian degeneration, in which the part of the axon distal to the break degrades, takes place within one to two days after injury. (wikipedia.org)
  • In the absence of NGF, inhibition of glycolysis along distal axons results in axon degeneration independent of cell death. (jneurosci.org)
  • These data indicate that the glycolytic pathway is operative in distal axons and contributes to the rate of axon extension and growth cone dynamics in the presence of NGF and that, in the absence of NGF, the axonal glycolytic pathway is required for axon maintenance. (jneurosci.org)
  • Two recent articles published by Needleman Center researchers dissect the structure of SARM1, an enzyme responsible for initiating programmed axon degeneration, and shed light on how the balance of key cellular metabolites regulate its activity. (wustl.edu)
  • This results in free-radical formation and the selective degeneration of the serotonergic axons and axon terminals. (erowid.org)
  • In the visual pathway of patients with multiple sclerosis (MS), the inner nuclear layer (INL) of the retina is a tight barrier for retrograde trans-synaptic degeneration. (amsterdamumc.org)
  • In this observational, retrospective cross-sectional study, segmented macular spectral domain optical coherence tomography (OCT) volume scans were reviewed to investigate if this observation also holds true for anterograde trans-synaptic degeneration. (amsterdamumc.org)
  • Significant thinning was found in all retinal layers in patients with outer retinal diseases compared with the healthy controls, while there was no significant attenuation of the outer retina in patients with MS. In contrast to the tight barrier function observed with retrograde trans-synaptic degeneration, the INL appears to be more permissive for the propagation of anterograde trans-synaptic degeneration. (amsterdamumc.org)
  • In particular, this project investigates the potential role of trans-synaptic degeneration in multiple sclerosis. (edu.au)
  • The myelin-associated proteins Nogo, myelin-associated glycoprotein (MAG), and oligodendrocyte myelin glycoprotein (OMgp) are known to inhibit axonal plasticity, and thus targeting the inhibitory pathways they participate in is a potential means of promoting plasticity and functional recovery. (jneurosci.org)
  • The extent to which this axonal reorganization occurred was far greater in mice lesioned during early postnatal days than in mice lesioned at an age when myelin had begun to form. (jneurosci.org)
  • This suggests that myelin-associated proteins might limit axonal remodeling in vivo . (jneurosci.org)
  • It has been believed that an inhibitory property of myelin is one of the main factors limiting axonal growth and plasticity ( Yiu and He, 2006 ). (jneurosci.org)
  • These results suggest that PirB might contribute to the inhibition of axonal plasticity by myelin in vivo . (jneurosci.org)
  • Myelin insulation as a risk factor for axonal degeneration in autoimmune demyelinating disease. (mpg.de)
  • 2012) Glycolytic oligodendrocytes maintain myelin and long-term axonal integrity. (als.net)
  • 1998) Axonal swellings and degeneration in mice lacking the major proteolipid of myelin. (als.net)
  • Our work further identifies cerebrospinal fluid neurofilament light and plasma neurofilament light as biomarkers of axonal degeneration in patients with mild cognitive impairment and Alzheimer's disease. (imrpress.com)
  • The axonal diameter correlates with the neurofilament (Nf) protein content, but there are no data on the retinal tissue concentration of Nfs. (lu.se)
  • Axonal degeneration, which contributes to functional impairment in several disorders of the nervous system, is an important target for neuroprotection. (lu.se)
  • The accomplishment of these aims will identify a role for the complement pathway in the A1 reactive astrocyte phenotype as well as in A1-mediated RGC degeneration. (brightfocus.org)
  • Several individual factors and subcellular events have been implicated in axonal degeneration, but researchers have so far been unable to identify an integrative signaling pathway activating this self-destructive process. (lu.se)
  • Activation of the necroptotic pathway early during injury-induced axonal degeneration was made evident by increased phosphorylation of the downstream effector MLKL. (lu.se)
  • Several in vitro studies have shown that the binding of HIV envelope protein Gp120 to chemokine receptors leads to activation of the caspase pathway and axonal degeneration. (medscape.com)
  • Optic nerve section in the newborn rat results in a rapid apoptotic degeneration of most axotomized retinal ganglion cells (RGCs). (nih.gov)
  • To distinguish between these two possibilities, we induced a reversible axonal transport blockade in the developing optic nerve by topical application of a local anesthetic (lidocaine). (nih.gov)
  • RIPK1 inhibition prevented mitochondrial fragmentation in vitro and in vivo, a typical feature of necrotic death, and inhibition of mitochondrial fission by Mdivi also resulted in reduced axonal loss in damaged nerves. (lu.se)
  • Furthermore, electrophysiological analysis demonstrated that inhibition of necroptosis delays not only the morphological degeneration of axons, but also the loss of their electrophysiological function after nerve injury. (lu.se)
  • Considering filament polymerization requires ATP, inhibition of glycolysis results in a paradoxical increase in axonal actin filament levels. (jneurosci.org)
  • Thus, both correct long-range signaling from the periphery and maintenance of the axonal network are paramount to neuronal function and survival. (oist.jp)
  • In the late 1960s, neurophysiologic testing allowed the classification of CMT into 2 groups, one with slow nerve conduction velocities and histologic features of a hypertrophic demyelinating neuropathy (hereditary motor and sensory neuropathy type 1 or CMT1) and another with relatively normal velocities and axonal and neuronal degeneration (hereditary motor and sensory neuropathy type 2 or CMT2). (medscape.com)
  • Autosomal recessive forms can be also divided into demyelinating (CMT4 or AR-CMT1) and axonal forms (AR-CMT2). (medscape.com)
  • Traditionally, CMT pathophysiology has been categorized into 2 processes: a predominant demyelinating process resulting in low conduction velocities (CMT1) and a predominant axonal process resulting in low potential amplitudes (CMT2). (medscape.com)
  • Demyelinating and axonal forms of Guillain-Barré syndrome (GBS) have both been described. (medscape.com)
  • This interaction then causes the acute demyelinating polyradiculoneuropathy or, particularly in cases involving C jejuni infection, an acute axonal degeneration. (medscape.com)
  • Its levels increase in cerebrospinal fluid (CSF) and blood proportionally to the degree of axonal damage in a variety of neurological disorders, including inflammatory, neurodegenerative, traumatic and cerebrovascular diseases. (bmj.com)
  • To investigate how A1 reactive astrocytes modulate the RGC axonal compartment, RGCs will be grown in microfluidic devices and cultured with astrocytes within the axonal compartment (Aim 2). (brightfocus.org)
  • To explore if modulation of A1 astrocytes results in protective effects upon RGCs, RGCs will be grown with C3 shRNA-transduced astrocytes within the axonal compartment (Aim 3). (brightfocus.org)
  • We found that axonal transport blockade induced only a limited number of pyknotic RGCs. (nih.gov)
  • We conclude that the rapid degeneration of RGCs after axotomy can be ascribed only partly to the loss of retrogradely transported trophic factors. (nih.gov)
  • To determine if Aβ accumulation is directly involved in degeneration of MAergic axons, we examined the effects of passive anti-Aβ antibody (7B6) administration on Aβ pathology and MAergic degeneration in AβPPswe/PS1dE9 mice. (umn.edu)
  • Besides mechanical breakage of the axonal cytoskeleton, DAI pathology also includes secondary physiological changes, such as interrupted axonal transport, progressive swellings known as axonal varicosities, and degeneration. (wikipedia.org)
  • The Shaffer Grant allowed me to focus on understanding the molecular signaling pathways controlling axonal degeneration. (glaucoma.org)
  • Our results demonstrate that axonal degeneration proceeds by necroptosis, thus defining a novel mechanistic framework in the axonal degenerative cascade for therapeutic interventions in a wide variety of conditions that lead to neuronal loss and functional impairment. (lu.se)
  • In the management of neurological diseases, the identification and quantification of axonal damage could allow for the improvement of diagnostic accuracy and prognostic assessment. (bmj.com)
  • Furthermore, as a quantitative measure of the ongoing axonal injury, the increase in NfL levels could have a prognostic value in a variety of neurological diseases. (bmj.com)
  • The RNFL is composed of unmyelinated axons, and measuring RNFL thickness is a viable method to monitor axonal loss reflecting disease progression. (tjn.org.tr)
  • The data suggest that up-regulation of Nf expression in the retinal ganglion cells precedes development of RNFL atrophy and plausibly explains the transient increase of axonal diameter and RNFL thickening. (lu.se)
  • Diffuse axonal injury (DAI) is a brain injury in which scattered lesions occur over a widespread area in white matter tracts as well as grey matter. (wikipedia.org)
  • Even though muscle development is unaffected by nicotine exposure in sop mutants, motoneuron axonal pathfinding errors still occur in these mutants, indicating a direct effect of nicotine exposure on nervous system development. (oregonstate.edu)
  • Here, we determined whether blocking PirB activity enhances axonal reorganization and functional recovery after cortical injury. (jneurosci.org)
  • Our results suggest that blocking the function of PirB is not sufficient to promote axonal reorganization or functional recovery after cortical injury. (jneurosci.org)
  • In the present study, we aimed to reveal whether blocking the function of PirB enhances axonal remodeling and functional recovery after cortical injury. (jneurosci.org)
  • The acute motor axonal neuropathy (AMAN) subtype of GBS is a purely motor disorder that is more prevalent amongst pediatric age groups. (medscape.com)
  • Yield of next-generation neuropathy gene panels in axonal neuropathies. (cdc.gov)
  • However, even for CMT1 a heated debate has focused on the relative contribution of axonal versus demyelinative damage to the disease manifestations and progression. (medscape.com)
  • Concussion may be a milder type of diffuse axonal injury. (wikipedia.org)
  • This bulb is called a "retraction ball", the histological hallmark of diffuse axonal injury. (wikipedia.org)
  • To address this question, we examined the effects of Aβ antibodies in the recently described monoaminergic (MAergic) axonal degeneration in AβPPswe/PS1dE9 mice. (umn.edu)
  • Histological analyses of the lumbosacral spinal cord revealed that Bxt markedly delayed the early motor-neuron degeneration occurring at presymptomatic stages in ALS-transgenic mice. (frontiersin.org)
  • Axonal transport continues up to the point of the break in the cytoskeleton, but no further, leading to a buildup of transport products and local swelling at that point. (wikipedia.org)
  • Together with Misgeld's research group, they observed axonal organelle transport in living tissue in real time - and in a way that enabled them to track the movement of individual mitochondria - using a novel imaging approach that involves transgenic labeling. (mytum.de)
  • This suggests that, at least for ALS, axonal organelle transport may be an unsuitable therapeutic target. (mytum.de)
  • Moreover, in more general biological terms, our results also speak to the relationship between axonal transport disruptions and degeneration - which might not be as tight as we assumed. (mytum.de)
  • Degeneration of these cells was completely prevented by inhibiting protein synthesis during lidocaine application. (nih.gov)
  • Nicotinic receptors mediate changes in spinal motoneuron development and axonal pathfinding in embryonic zebrafish exposed to nicotine. (oregonstate.edu)
  • If so, PirB might be an attractive target of therapies aiming to enhance axonal plasticity after brain injury. (jneurosci.org)
  • Your main project will involve the characterisation of mutant lines identified in a forward genetic screen for genes required for axonal maintenance (unpublished). (academictransfer.com)
  • This massive process of neuronal death has been ascribed mainly to the interruption of a trophic factor supply from target structures rather than to the axonal damage per se. (nih.gov)
  • Indeed, many neurodegenerative diseases display a "dying back" phenomenon, whereby more distant axonal segments are the first to be affected in the development of the disease. (oist.jp)
  • Further, degeneration of neuromuscular junctions, of synapses, and of axonal regions are features of SMA disease. (nih.gov)
  • Through pharmacological and genetic approaches, we tested whether necroptosis, a regulated cell-death mechanism implicated in the pathogenesis of several neurodegenerative diseases, is involved in axonal degeneration. (lu.se)