Results 61 to 70 of about 10,727,728 (294)

Trehalose alleviates the phenotype of Machado–Joseph disease mouse models [PDF]

open access: yes, 2020
Machado-Joseph disease (MJD), also known as spinocerebellar ataxia type 3, is the most common of the dominantly inherited ataxias worldwide and is characterized by mutant ataxin-3 aggregation and neuronal degeneration.
Cavadas, Cláudia   +10 more
core   +1 more source

Viewpoint: spinocerebellar ataxias as diseases of Purkinje cell dysfunction rather than Purkinje cell loss

open access: yesFrontiers in Molecular Neuroscience, 2023
Spinocerebellar ataxias (SCAs) are a group of hereditary neurodegenerative diseases mostly affecting cerebellar Purkinje cells caused by a wide variety of different mutations. One subtype, SCA14, is caused by mutations of Protein Kinase C gamma (PKCγ), the dominant PKC isoform present in Purkinje cells. Mutations in the pathway in which PKCγ is active,
Josef P. Kapfhammer, Etsuko Shimobayashi
openaire   +3 more sources

Clinical Spectrum and Outcomes of SOX1 Antibody‐Associated Paraneoplastic Neurological Syndromes: A Chinese Cohort Study

open access: yesAnnals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Background SOX1 antibody‐positive paraneoplastic neurological syndromes (PNS) exhibit significant population‐specific clinical heterogeneity. While Western cohorts predominantly manifest Lambert‐Eaton myasthenic syndrome (65%–80%), comprehensive clinical characterization and treatment response data in Asian populations remain critically ...
Jin‐Long Ye   +11 more
wiley   +1 more source

Investigating the Contribution of BK Channel in the Firing Activity of Cerebellar Purkinje Cells Using Computer Simulation

open access: yesمجله دانشکده پزشکی اصفهان, 2012
Background: BK channels are highly conductive calcium (Ca2+)-activated potassium (K+) channels. These channels are available on the membrane of many types of neurons including cerebellar Purkinje cells. Several experimental studies have used iberiotoxin,
Hassan Zolfeghar Didani   +2 more
doaj   +2 more sources

Motor context dominates output from purkinje cell functional regions during reflexive visuomotor behaviours

open access: yeseLife, 2019
The cerebellum integrates sensory stimuli and motor actions to enable smooth coordination and motor learning. Here we harness the innate behavioral repertoire of the larval zebrafish to characterize the spatiotemporal dynamics of feature coding across ...
Laura D Knogler   +2 more
doaj   +1 more source

PSD-95 Is Associated with the Postsynaptic Density and Not with the Presynaptic Membrane at Forebrain Synapses [PDF]

open access: yes, 1996
PSD-95, a prominent protein component of the postsynaptic density (PSD) fraction from rat forebrain, has been localized by light microscopy to dendrites of hippocampal neurons (Cho et al., 1992) and to the presynaptic plexus of cerebellar basket cells ...
Hunt, Carol A.   +2 more
core   +1 more source

Clinical Practice Guideline for Evaluation and Management of Peripheral Nervous System Manifestations in Sjögren's Disease

open access: yesArthritis Care &Research, Accepted Article.
Objectives Sjögren's disease is an autoimmune disorder that can impact multiple organ systems, including the peripheral nervous system (PNS). PNS manifestations, which can exist concurrently, include mononeuropathies, polyneuropathies, and autonomic nervous system neuropathies. To help patients and providers in the decision‐making process, we developed
Anahita Deboo   +19 more
wiley   +1 more source

Mesenchymal stem cells rescue Purkinje cells and improve motor functions in a mouse model of cerebellar ataxia

open access: yesNeurobiology of Disease, 2010
Mesenchymal stem cells have been proven to be potentially effective in the treatment of a large variety of diseases, including neurodegenerative disorders.
Jonathan Jones   +5 more
doaj   +1 more source

Purkinje cell loss in essential tremor [PDF]

open access: yesMovement Disorders, 2014
The recent study published by Symanski et al.1 reports similar Purkinje cell counts in a group of “essential tremor” cases compared to a group of controls. There are a number of major methodological problems with the design of the study and these problems cast doubt on the validity of the results that are reported.
Elan D, Louis, Phyllis L, Faust
openaire   +2 more sources

Wearable and Implantable Devices for Continuous Monitoring of Muscle Physiological Activity: A Review

open access: yesAdvanced Science, EarlyView.
Recent advances in materials and device engineering enable continuous, real‐time monitoring of muscle activity via wearable and implantable systems. This review critically summarizes emerging technologies for tracking electrophysiological, biomechanical, and oxygenation signals, outlines fundamental principles, and highlights key challenges and ...
Zhengwei Liao   +4 more
wiley   +1 more source

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