Results 61 to 70 of about 10,727,728 (294)
Trehalose alleviates the phenotype of Machado–Joseph disease mouse models [PDF]
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
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
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
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
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]
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
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 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]
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
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

