Results 161 to 170 of about 48,590 (271)
Repositioning of vemurafenib and palbociclib as spleen tyrosine kinase inhibitors for the treatment of neuroinflammation in Ataxia–Telangiectasia. ABSTRACT Ataxia–Telangiectasia (AT) is a rare neurodegenerative disorder characterized by progressive neuronal loss and chronic neuroinflammation.
Alessia Romano +3 more
wiley +1 more source
Abstract After treatment with new disease‐modifying therapies, cognitive and neurodevelopmental aspects have been observed in individuals with spinal muscular atrophy (SMA). Emerging evidence suggests that children with SMA type 1 may experience cognitive, language, and behavioural delays, with reported rates of neurodevelopmental difficulties ranging ...
Giorgia Coratti +2 more
wiley +1 more source
Steady-state Purkinje cell activity has limited predictive power for cerebellar output in disease. [PDF]
Lyon AM +2 more
europepmc +1 more source
ABSTRACT Objectives To expand the RORA mutational and clinical spectrum by reporting a novel hinge‐region variant associated with an adult progressive phenotype and a marked therapeutic response to valproate. Methods We describe a 61‐year‐old woman with lifelong intellectual disability who developed subacute severe motor deterioration in adulthood ...
V. Busco +6 more
wiley +1 more source
Climbing fibres recruit disinhibition to enhance Purkinje cell calcium signals. [PDF]
Santos-Valencia F +11 more
europepmc +1 more source
Variability in intracellular localization of D‐amino acid oxidase in choroid plexus epithelial cells
D‐amino acid oxidase (DAO) in choroid plexus epithelial cells (CPECs) shows vesicle‐like localization by histological and super‐resolution analyses. DAO colocalizes with peroxisomal, Golgi, endosomal, lysosomal, autophagosomal, and exosomal markers, indicating diverse subcellular distribution. This suggests DAO is transported within CPECs to metabolize
Koji Ono +3 more
wiley +1 more source
Oligodendrocyte dysfunction contributes to motor deficits and Purkinje cell axonopathy in spinocerebellar ataxia type 1. [PDF]
Lee C +9 more
europepmc +1 more source
Splice Type‐Specific Effects of Gαo Subunits on Cerebellar Anatomy and Synapse Formation
Heterotrimeric Go alpha subunits are widely expressed in the brain to act as molecular switches. In this study we show that Gαo1 and Gαo2 affect cerebellar morphology and synapse formation in an opposite manner. While knockout of Gαo1 results in cerebellar hypoplasia and reduced synaptic input to Purkinje cells by VGLUT2‐positive brainstem climbing ...
Markus Höltje +2 more
wiley +1 more source
Pregabalin Depresses Cerebellar Parallel Fiber-Purkinje Cell Synaptic Transmission by Modulating Glun2a-Containing Nmda Receptors in Mice In Vitro. [PDF]
Li MR +8 more
europepmc +1 more source
Regeneration of the lizard heart after cryoinjury
Abstract Lizards are renowned for their tremendous potential to heal tissues and organs after injury, but little is known about myocardial regeneration in reptiles generally. Here, we study cardiac regeneration in the leopard gecko (Eublepharis macularius) to fill the knowledge gap between traditional models of poikilothermic (zebrafish) and ...
Martina Gregorovicova +5 more
wiley +1 more source

