• Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that is frequently linked to microtubule abnormalities and mitochondrial trafficking defects. (en-journal.org)
  • Several studies have identified oxidative stress, glutamate excitotoxicity, apoptosis, neurofilament dysfunction, protein misfolding and aggregation, impairment of RNA processing, disrupted axonal transport, endosomal trafficking dysfunction, inflammation, and mitochondrial impairment as the molecular pathways which lead to the disease and indicate ALS pathogenesis. (encyclopedia.pub)
  • However, other molecular mechanisms underlying mitochondrial defect in diabetic retinal neurodegeneration remain to be elucidated. (aging-us.com)
  • Rescue of β-catenin via ectopic expression of β-catenin with a recombinant adenoviral vector, or via GSK3β inhibition by a targeted si- GSK3β , through intravitreal administration, abrogated the oxidative stress-derived mitochondrial defect and synaptic neurodegeneration in diabetic RGCs. (aging-us.com)
  • Several APOE isoforms with major structural differences were discovered and shown to influence the brain lipid transport, glucose metabolism, neuronal signaling, neuroinflammation, and mitochondrial function. (dovepress.com)
  • Overexpression of the RAPGEF2 mutant in Drosophila motor neurons reduces the stability of axonal microtubules and disrupts the distribution of mitochondria to distal axons and neuromuscular junction (NMJ) synapses. (en-journal.org)
  • Note how quickly DRG neurons extend axonal processes. (biomedcentral.com)
  • Apolipoprotein E (APOE) is a lipid-transport protein abundantly expressed in most neurons in the central nervous system. (dovepress.com)
  • Eukaryotic cells transport packets of components to particular intracellular locations by attaching them to molecular motors that haul them along microtubules and actin filaments. (wikipedia.org)
  • Since intracellular transport heavily relies on microtubules for movement, the components of the cytoskeleton play a vital role in trafficking vesicles between organelles and the plasma membrane by providing mechanical support. (wikipedia.org)
  • 10) suggesting that excess tau in the absence of fibrillary tau inclusions "clogs" microtubules (MTs) and impedes axonal transport. (alzforum.org)
  • We have recently shown that aberrant hyperphosphorylation of the microtubule-associated protein tau inhibits microtubule-dependent axonal/dendritic trafficking of mitochondria, thereby depriving synapses of mitochondria and causing the starvation of synapses, leading to synapse loss and dysfunction of RGCs in diabetic retinae [ 6 ]. (aging-us.com)
  • Indeed, there is growing evidence that failed axonal transport might be the underlying basis for several neurodegenerative diseases in addition to tauopathies (8). (alzforum.org)
  • Long-distance axonal trafficking plays a critical role in neuronal function and transport defects have been linked to neurodegenerative disorders. (mpg.de)
  • Giant axonal neuropathy (GAN) is a rare, autosomal recessive, progressive neurodegenerative disorder affecting both the central and peripheral nervous systems. (journalmc.org)
  • First identified in 1972, giant axonal neuropathy (GAN) is a rare, autosomal recessive, progressive neurodegenerative disorder affecting both the central and peripheral nervous systems. (journalmc.org)
  • Phosphatidylserine Ameliorates Neurodegenerative Symptoms and Enhances Axonal Transport in a Mouse Model of Familial Dysautonomia. (genscript.com)
  • This study demonstrated strong topographical spread of neurodegeneration along recognized neural projections and showed that myelin and glial pathology precedes axonal loss in the process, suggesting that the mechanism of trans-synaptic damage may be at least partially mediated by glial components at the cellular level. (edu.au)
  • Retrograde transport of Akt by a neuronal Rab5-APPL1 endosome. (mpg.de)
  • Here, we characterized the motility of Rab5 endosomes in primary cultures of mouse hippocampal pyramidal cells by live-cell imaging and showed that they exhibit bi-directional long-range motility in axons, with a strong bias toward retrograde transport. (mpg.de)
  • Further analysis of APPL1-positive endosomes showed that, similar to Rab5-endosomes, they display more frequent long-range retrograde than anterograde movement, with the endosomal levels of APPL1 correlated with faster retrograde movement. (mpg.de)
  • Interestingly, APPL1-endosomes transport the neurotrophin receptor TrkB and mediate retrograde axonal transport of the kinase Akt1. (mpg.de)
  • These vesicles will typically execute cargo loading and vesicle budding, vesicle transport, the binding of the vesicle to a target membrane and the fusion of the vesicle membranes to target membrane. (wikipedia.org)
  • Small membrane bound vesicles responsible for transporting proteins from one organelle to another are commonly found in endocytic and secretory pathways. (wikipedia.org)
  • In order for the transport vesicle to accurately undergo a fusion event, it must first recognize the correct target membrane then fuse with that membrane. (wikipedia.org)
  • C. A slow depolarization occurs when Na+ is transported out of the cell and K+ moves back in, resulting in resting membrane potential. (nursingessay24x7.com)
  • Although some substances have a particular affinity to certain areas of the nervous system, most neurotoxins have widespread effects, targeting cell processes involved in membrane transport, internal cellular chemical reactions, liberation of secretory substances, and so on. (iloencyclopaedia.org)
  • Action potential propagation along an axon is electrical, caused by the exchanges of sodium and potassium ions across the axonal membrane. (msdmanuals.com)
  • We conclude that Rab5-APPL1 endosomes exhibit the hallmarks of axonal signaling endosomes to transport Akt1 in hippocampal pyramidal cells. (mpg.de)
  • However, the synaptic phenotype in the rab3-GEF mutant cannot be fully explained by defective transport and loss of GEF activity. (eneuro.org)
  • This study makes increasingly clear that there is a critical need for more studies of this kind to understand how perturbations in tau expression levels or tau pathologies are linked to axonal transport failure and tau-mediated neurodegeneration in Alzheimer disease (AD) and related tauopathies. (alzforum.org)
  • As a result, this leaves less normal tau available to stabilize MTs, and, when MTs are destabilized, this compromises intraneuronal transport leading to neurodegeneration. (alzforum.org)
  • Rab proteins on the surface of the transport vesicle are responsible for aligning with the complementary tethering proteins found on the respective organelle's cytosolic surface. (wikipedia.org)
  • Intracellular transport is the movement of vesicles and substances within a cell. (wikipedia.org)
  • Intracellular transport is required for maintaining homeostasis within the cell by responding to physiological signals. (wikipedia.org)
  • Intracellular transport is unique to eukaryotic cells because they possess organelles enclosed in membranes that need to be mediated for exchange of cargo to take place. (wikipedia.org)
  • Intracellular transport is an overarching category of how cells obtain nutrients and signals. (wikipedia.org)
  • One very well understood form of intracellular transport is known as endocytosis. (wikipedia.org)
  • Phagocytosis is of great importance to intracellular transport because once a substance is deemed harmful and engulfed in a vesicle, it can be trafficked to the appropriate location for degradation. (wikipedia.org)
  • Finally, increasing microtubule stability through pharmacological inhibition of histone deacetylase 6 (HDAC6) rescues defects in the intracellular distribution of mitochondria and BAX. (en-journal.org)
  • D. The fast sodium channels in the cellular membranes close, causing an abrupt decrease in intracellular positivity. (nursingessay24x7.com)
  • Chédiak-Higashi syndrome (CHS) is an autosomal recessive immunodeficiency disorder characterized by abnormal intracellular protein transport. (medscape.com)
  • Various lines of evidence suggest that the small GTPase Rab5 plays a role in neuronal signaling via early endosomal transport. (mpg.de)
  • versus 20 controls), that there are measurable topographic changes in the posterior visual pathways corresponding to the primary optic nerve defects. (edu.au)
  • Glaucoma suspect describes a person with one or more risk factors that may lead to glaucoma, but this individual does not have definite glaucomatous optic nerve damage or visual field defect. (medscape.com)
  • [ 5 ] The blood supply to the optic nerve, the axonal or ganglion cell metabolism, and the lamina cribrosa extracellular matrix may play a role. (medscape.com)
  • Similarly, many patients with other risk factors, such as suspicious optic nerve appearance, nerve fiber layer defects, and family history of glaucoma without definite glaucomatous damage, are observed. (medscape.com)
  • A 31-year-old woman with a congenital heart defect reports episodes of light-headedness and syncope, with occasional palpitations. (nursingessay24x7.com)
  • Postnatal transgenic expression of Cntnap1 in the mutant backgrounds rescues the phenotypes and restores the organization of axonal domains with improved motor function. (bvsalud.org)
  • Additionally, leucine substitutions at this position caused similar signaling defects in the follicle-stimulating hormone receptor (FSHR), Galanin receptor (GALR1), AKH receptor (AKHR), corazonin receptor (CRZR), and muscarinic acetylcholine receptor (mACHR1) . (sdbonline.org)
  • More often it is due to a resistance to the action of insulin either due to a receptor/post receptor defect or an imbalance between insulin and its counter regulatory hormones. (nurseinfo.in)
  • showed for the first time that the development of fibrillary tau pathology was linked to MT loss, impaired fast axonal transport (FAT) using the Lasek et al. (alzforum.org)
  • The encoded protein is involved in lipid metabolism, and ablation of this gene results in defects in triglyceride metabolism. (genscript.com)
  • In Griscelli syndrome type 1, a defect in the myosin Va gene leads to the pigment dilution and neurological sequelae. (medscape.com)
  • Children with a defect in the MYO5A gene (Griscelli syndrome type 1) develop neurologic problems but no immunologic problems. (medscape.com)
  • Griscelli syndrome type 2 is caused by a defect in the RAB27A gene, which affects a melanosome-anchoring complex in melanocytes, affecting release of cytolytic granules from T cells and natural killer cells. (medscape.com)
  • The primary defect is a mutation in the GAN gene, which codes for the protein, gigaxonin. (journalmc.org)
  • The Contactin-associated protein 1 (Cntnap1) mouse mutants fail to establish proper axonal domains in myelinated axons. (bvsalud.org)
  • These two genetic defects result in both similar and distinct physical and pathologic findings. (medscape.com)
  • Axonal transport rates in vivo are unaffected by tau deletion or overexpression in mice. (alzforum.org)
  • report elegant studies of axonal transport in vivo using tau transgenic and tau knockout mice that overexpress human tau isoforms or completely lack tau expression, respectively. (alzforum.org)
  • These studies sought to elucidate the consequences of too much tau or a complete lack of tau on axonal transport in living mice. (alzforum.org)
  • Prevention of these abnormal changes in tau could inhibit the signaling cascade that leads to defects in FAT in patients with tauopathies (diseases like AD, CBD, and PSP). (brightfocus.org)
  • The coordinated growth and development of synapses is critical for all aspects of neural circuit function and mutations that disrupt these processes can result in various neurological defects. (sdbonline.org)
  • Among numerous physiological tasks, the vascular network transports oxygen and nutrients and eliminates waste products, all critical for organ survival and cellular homeostasis. (elifesciences.org)
  • Transport vesicles are small structures within the cell consisting of a fluid enclosed by a lipid bilayer that hold cargo. (wikipedia.org)
  • There are many inherited and acquired clinical disorders caused by a defect in upper or lower motor neurone or the peripheral nerve. (musculoskeletalkey.com)
  • Both the axonal type and the demyelinating type of peripheral neuropathy associated with CHS have been reported. (medscape.com)
  • Various glia-derived proteoglycans interact with NR axonal proteins to sustain NR function. (bvsalud.org)
  • The defect in the gigaxonin system leads to the aberrant accumulation of cytoskeletal-associated proteins (neurofilaments) due to defective function of the ubiquitin-proteasome system (UPS). (journalmc.org)
  • it is a multifaceted process which utilizes transport vesicles. (wikipedia.org)
  • The process is both faster and less technically challenging than extracting the ganglia from the in situ column after performing a dorsal laminectomy. (biomedcentral.com)
  • Three of the 200 transport process PDTC and 200 industry germany field forms fed been at 47 Solutions when three opportunities in the 200 iron argument PDTC intensity led to continue altered from the observation volcanic to supplementary sector AElig drug-delivery and infrastructure of their features, and at that degree, one contradictory 200 shophouse money PDTC manuscript with the lowest delivery distribution was resulted along with found names. (ogtechnology.com)
  • SNAREs are small, tail-anchored proteins which are often post-translationally inserted into membranes that are responsible for the fusion event necessary for vesicles to transport between organelles in the cytosol. (wikipedia.org)
  • This is a most welcome study by the Nixon lab, which has made important contributions to the understanding of axonal transport dynamics for over 2 decades. (alzforum.org)
  • A significant anisotropic increase of water diffusion was detected in both patient cohorts in the optic radiations, characterized by changes in perpendicular (radial) diffusivity (a measure of myelin integrity) that extended more posteriorly than those observed in parallel (axial) diffusivity (reflecting axonal integrity). (edu.au)
  • This method of transport is largely intercellular in lieu of uptake of large particles such as bacteria via phagocytosis in which a cell engulfs a solid particle to form an internal vesicle called a phagosome. (wikipedia.org)
  • Outbound proteins from the endoplasmic reticulum will bud off into transport vesicles that travel along the cell cortex to reach their specific destinations. (wikipedia.org)
  • We offer real benefits to our authors, including fast-track processing of papers. (dovepress.com)