Results 131 to 140 of about 10,727,728 (294)

The endocytic adaptor AP-2 maintains Purkinje cell function by balancing cerebellar parallel and climbing fiber synapses

Cell Reports
Summary: The loss of cerebellar Purkinje cells is a hallmark of neurodegenerative movement disorders, but the mechanisms remain enigmatic. We show that endocytic adaptor protein complex 2 (AP-2) is crucial for Purkinje cell survival. Using mouse genetics,
Marianna Tolve, Janine Tutas, Ebru Özer-Yildiz, Ines Klein, Anne Petzold, Veronika J. Fritz, Melina Overhoff, Quinn Silverman, Ellie Koletsou, Filip Liebsch, Guenter Schwarz, Tatiana Korotkova, Silvana Valtcheva, Graziana Gatto, Natalia L. Kononenko   +14 more
doaj   +1 more source

Increased protein kinase C gamma activity induces Purkinje cell pathology in a mouse model of spinocerebellar ataxia 14

Neurobiology of Disease, 2014
Spinocerebellar ataxias (SCAs) are hereditary diseases leading to Purkinje cell degeneration and cerebellar dysfunction. Most forms of SCA are caused by expansion of CAG repeats similar to other polyglutamine disorders such as Huntington's disease.
Jingmin Ji, Melanie L. Hassler, Etsuko Shimobayashi, Nagendher Paka, Raphael Streit, Josef P. Kapfhammer   +5 more
doaj   +1 more source

Cognitive and neurodevelopmental disorders in spinal muscular atrophy type I at the time of disease‐modifying therapies

Developmental Medicine &Child Neurology, EarlyView.
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, Bianca Buchignani, Eugenio Mercuri   +2 more
wiley   +1 more source

Human neuronal stargazin-like proteins, gamma(2), gamma(3) and gamma(4); an investigation of their specific localization in human brain and their influence on Ca(V)2.1 voltage-dependent calcium channels expressed in Xenopus oocytes [PDF]

, 2003
Background: Stargazin (gamma(2)) and the closely related gamma(3), and gamma(4) transmembrane proteins are part of a family of proteins that may act as both neuronal voltage-dependent calcium channel (VDCC) gamma subunits and transmembrane alpha-amino-3 ...
Clare, JJ, Dolphin, AC, Moss, FJ
core  

Treatment approaches in posthypoxic myoclonus: A narrative review with expert opinion

Epilepsia, EarlyView.
Abstract Acute posthypoxic myoclonus (PHM) is a neurological complication that typically emerges within 12–48 h following cardiac arrest, often in comatose patients. It can present as generalized, multifocal, or focal myoclonus and has traditionally been associated with poor prognosis.
Marina Romozzi, Federico Tosto, David García‐Azorín, Luigi Francesco Iannone, Catello Vollono, Paolo Calabresi, Juan Luis Alcala‐Zermeno, Michael R. Sperling   +7 more
wiley   +1 more source

Purkinje cells and their trees

The Lancet Neurology, 2021
Zoghbi, Huda Y, Mehta, Arpan R
openaire   +4 more sources

Newly identified properties of known pharmaceuticals and myxobacterial small molecules revealed by screening for autophagy modulators

The FEBS Journal, EarlyView.
Autophagy is a cellular degradation process involved in, for example, immune responses to pathogens and neurodegeneration. To identify modulators of autophagy, we developed a microscopy‐based screening assay and identified previously unknown autophagy‐modulating activities in known drugs and natural products from myxobacteria and fungi.
Janine Fichtner, Yan Yan Beer, H. G. Mauricio Ramm, Sabrina Mühlen, Frank Surup, Jennifer Herrmann, Toni Luise Meister, Stephanie Pfaender, Ursula Bilitewski, Mark Brönstrup, Rolf Müller, Manfred Wirth, Eeva‐Liisa Eskelinen, Ingo Schmitz   +13 more
wiley   +1 more source

Isoperimetric Partitioning: A New Algorithm for Graph Partitioning [PDF]

, 2003
Temporal structure is skilled, fluent action exists at several nested levels. At the largest scale considered here, short sequences of actions that are planned collectively in prefronatal cortex appear to be queued for performance by a cyclic competitive
Grady, Leo, Schwartz, Eric
core   +1 more source

Conditional deletion of the multiple sclerosis susceptibility gene ATXN1 identifies cell‐autonomous effects in the B‐cell compartment

The FEBS Journal, EarlyView.
Ataxin‐1 is a polyglutamine protein associated with the etiology of spinocerebellar ataxia type 1 (SCA1) that has been recently found implicated in the risk of developing the autoimmune disorder multiple sclerosis (MS). Here, we took a conditional knockout approach to ablate ataxin‐1 exclusively in the B‐cell compartment and we found that this protein ...
Jonathan Jacob Carver, Rachael Riley Denbrock, Cindy Carolina Martines, Alessandro Didonna   +3 more
wiley   +1 more source

Mild Cerebellar Neurodegeneration of Aged Heterozygous PCD Mice Increases Cell Fusion of Purkinje and Bone Marrow-Derived Cells

Cell Transplantation, 2012
Bone marrow-derived cells have different plastic properties, especially regarding cell fusion, which increases with time and is prompted by tissue injury.
David Díaz, Javier S. Recio, Eduardo Weruaga, Jose R. Alonso   +3 more
doaj   +1 more source