Explanipedia

Increased intrinsic membrane excitability is associated with olivary hypertrophy in spinocerebellar ataxia type 1 Open

Logan M Morrison, Haoran Huang, Hillary P. Handler, Min Fu, Deborah M. Jones , et al. · 2024

One of the characteristic regions of brainstem degeneration across multiple spinocerebellar ataxias (SCAs) is the inferior olive (IO), a medullary nucleus that plays a key role in motor learning. The vulnerability of IO neurons remains a p…

Increased intrinsic membrane excitability is associated with hypertrophic olivary degeneration in spinocerebellar ataxia type 1 Open

Logan M Morrison, Haoran Huang, Hillary P. Handler, Min Fu, David D. Bushart , et al. · 2023

One of the characteristic areas of brainstem degeneration across multiple spinocerebellar ataxias (SCAs) is the inferior olive (IO), a medullary nucleus that plays a key role in motor learning. In addition to its vulnerability in SCAs, the…

Vulnerability of Human Cerebellar Neurons to Degeneration in Ataxia-Causing Channelopathies Open

David D. Bushart, Vikram G. Shakkottai · 2022

Mutations in ion channel genes underlie a number of human neurological diseases. Historically, human mutations in ion channel genes, the so-called channelopathies, have been identified to cause episodic disorders. In the last decade, howev…

Preliminary Study of Vibrotactile Feedback during Home-Based Balance and Coordination Training in Individuals with Cerebellar Ataxia Open

Safa Jabri, David D. Bushart, Catherine Kinnaird, Tian Bao, Angel Bu , et al. · 2022

Intensive balance and coordination training is the mainstay of treatment for symptoms of impaired balance and mobility in individuals with hereditary cerebellar ataxia. In this study, we compared the effects of home-based balance and coord…

Compartmentalized Actions of the Plasminogen Activator Inhibitors, PAI-1 and Nsp, in Ischemic Stroke Open

Daniel Torrente, Enming J. Su, Linda Fredriksson, Mark Warnock, David D. Bushart , et al. · 2022

Tissue plasminogen activator (tPA) is a multifunctional protease. In blood tPA is best understood for its role in fibrinolysis, whereas in the brain tPA is reported to regulate blood-brain barrier (BBB) function and to promote neurodegener…

A Chlorzoxazone‐Baclofen Combination Improves Cerebellar Impairment in Spinocerebellar Ataxia Type 1 Open

David D. Bushart, Haoran Huang, Luke J. Man, Logan M Morrison, Vikram G. Shakkottai · 2020

Background A combination of central muscle relaxants, chlorzoxazone and baclofen (chlorzoxazone‐baclofen), has been proposed for treatment of cerebellar symptoms in human spinocerebellar ataxia. However, central muscle relaxants can worsen…

Altered Capicua expression drives regional Purkinje neuron vulnerability through ion channel gene dysregulation in spinocerebellar ataxia type 1 Open

Ravi Chopra, David D. Bushart, John P Cooper, Dhananjay Yellajoshyula, Logan M Morrison , et al. · 2020

Selective neuronal vulnerability in neurodegenerative disease is poorly understood. Using the ATXN1[82Q] model of spinocerebellar ataxia type 1 (SCA1), we explored the hypothesis that regional differences in Purkinje neuron degeneration co…

A chlorzoxazone-baclofen combination improves cerebellar impairment in spinocerebellar ataxia type 1 Open

David D. Bushart, Haoran Huang, Luke J. Man, Logan M Morrison, Vikram G. Shakkottai · 2020

Background A combination of central muscle relaxants, chlorzoxazone and baclofen (chlorzoxazone-baclofen), has been proposed for treatment of cerebellar symptoms in human spinocerebellar ataxia (SCA). However, central muscle relaxants can …

Antisense Oligonucleotide Therapy Targeted Against ATXN3 Improves Potassium Channel–Mediated Purkinje Neuron Dysfunction in Spinocerebellar Ataxia Type 3 Open

David D. Bushart, Annie J. Zalon, Hongjiu Zhang, Logan M Morrison, Yuanfang Guan , et al. · 2020

Altered Capicua expression drives regional Purkinje neuron vulnerability through ion channel gene dysregulation in Spinocerebellar ataxia type 1 Open

Ravi Chopra, David D. Bushart, John P Cooper, Dhananjay Yellajoshyula, Logan M Morrison , et al. · 2020

Selective neuronal vulnerability in neurodegenerative disease is poorly understood. Using the ATXN1[82Q] model of spinocerebellar ataxia type 1 (SCA1), we explored the hypothesis that regional differences in Purkinje neuron degeneration co…

Nicotinamide Pathway-Dependent Sirt1 Activation Restores Calcium Homeostasis to Achieve Neuroprotection in Spinocerebellar Ataxia Type 7 Open

Colleen A. Stoyas, David D. Bushart, Paweł M. Świtoński, Jacqueline M. Ward, Akshay Alaghatta , et al. · 2019

Synthetic high-density lipoprotein nanoparticles for the treatment of Niemann–Pick diseases Open

Mark L. Schultz, Maria V. Fawaz, Ruth D. Azaria, Todd Hollon, Elaine A. Liu , et al. · 2019

Antisense oligonucleotide therapy rescues aggresome formation in a novel spinocerebellar ataxia type 3 human embryonic stem cell line Open

Lauren R. Moore, Laura Keller, David D. Bushart, Rodrigo G. Delatorre, Duojia Li , et al. · 2019

Antisense oligonucleotide therapy rescues aggresome formation in a novel Spinocerebellar Ataxia type 3 human embryonic stem cell line Open

Lauren R. Moore, Laura Keller, David D. Bushart, Rodrigo G. Delatorre, Duojia Li , et al. · 2019

Spinocerebellar ataxia type 3 (SCA3) is a fatal, late-onset neurodegenerative disorder characterized by selective neuropathology in the brainstem, cerebellum, spinal cord, and substantia nigra. Here, we characterize the first NIH-approved …

Dendritic potassium channel dysfunction may contribute to dendrite degeneration in spinocerebellar ataxia type 1 Open

Ravi Chopra, David D. Bushart, Vikram G. Shakkottai · 2018

Purkinje neuron dendritic degeneration precedes cell loss in cerebellar ataxia, but the basis for dendritic vulnerability in ataxia remains poorly understood. Recent work has suggested that potassium (K+) channel dysfunction and consequent…

Targeting potassium channels to treat cerebellar ataxia Open

David D. Bushart, Ravi Chopra, Vikrant Singh, Geoffrey G. Murphy, Heike Wulff , et al. · 2018

Objective Purkinje neuron dysfunction is associated with cerebellar ataxia. In a mouse model of spinocerebellar ataxia type 1 ( SCA 1), reduced potassium channel function contributes to altered membrane excitability resulting in impaired P…

The Contribution of Calcium-Activated Potassium Channel Dysfunction to Altered Purkinje Neuron Membrane Excitability in Spinocerebellar Ataxia Open

David D. Bushart · 2018

Spinocerebellar ataxias (SCA) are a family of dominantly-inherited neurodegenerative disorders which affect movement and coordination. Patients experience the shared features of cerebellar ataxia, characterized by uncoordinated limb moveme…

Precision medicine in spinocerebellar ataxias: treatment based on common mechanisms of disease. Open

David D. Bushart, Geoffrey G. Murphy, Vikram G. Shakkottai · 2016

Spinocerebellar ataxias (SCAs) are a heterogeneous group of dominantly inherited neurodegenerative disorders affecting the cerebellum and its associated pathways. There are no available symptomatic or disease-modifying therapies available …

David D. Bushart YOU? Author Swipe