Sriram Jayabal
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View article: TRanslational Initiative to DE‐risk NeuroTherapeutics (TRIDENT) – A revolutionary pre‐clinical approach to maximize the predictive validity of pre‐clinical evaluation
TRanslational Initiative to DE‐risk NeuroTherapeutics (TRIDENT) – A revolutionary pre‐clinical approach to maximize the predictive validity of pre‐clinical evaluation Open
Background Neurodegenerative diseases such as Alzheimer's Disease (AD), Frontotemporal Dementia (FTD), Dementia with Lewy Bodies (DLB), Parkinson's Disease (PD), and Multiple System Atrophy (MSA), are complex and poorly understood conditio…
View article: Activation of TrkB-Akt signaling rescues deficits in a mouse model of SCA6
Activation of TrkB-Akt signaling rescues deficits in a mouse model of SCA6 Open
Spinocerebellar ataxia type 6 (SCA6) is a neurodegenerative disease resulting in motor coordination deficits and cerebellar pathology. Expression of brain-derived neurotrophic factor (BDNF) is reduced in postmortem tissue from SCA6 patient…
View article: Restoration of BDNF-TrkB signaling rescues deficits in a mouse model of SCA6
Restoration of BDNF-TrkB signaling rescues deficits in a mouse model of SCA6 Open
Spinocerebellar ataxia type 6 (SCA6) is a neurodegenerative disease resulting in motor coordination deficits and cerebellar pathology. Expression of brain-derived neurotrophic factor (BDNF) is reduced in several neurodegenerative diseases,…
View article: Transient Developmental Purkinje Cell Axonal Torpedoes in Healthy and Ataxic Mouse Cerebellum
Transient Developmental Purkinje Cell Axonal Torpedoes in Healthy and Ataxic Mouse Cerebellum Open
Information is carried out of the cerebellar cortical microcircuit via action potentials propagated along Purkinje cell axons. In several human neurodegenerative diseases, focal axonal swellings on Purkinje cells - known as torpedoes - hav…
View article: Transient cerebellar alterations during development prior to obvious motor phenotype in a mouse model of spinocerebellar ataxia type 6
Transient cerebellar alterations during development prior to obvious motor phenotype in a mouse model of spinocerebellar ataxia type 6 Open
Key points Spinocerebellar ataxia type 6 (SCA6) is a midlife‐onset neurodegenerative disease caused by a CACNA1A mutation; CACNA1A is also implicated in cerebellar development. We have previously shown that when disease symptoms are presen…
View article: 4-aminopyridine reverses ataxia and cerebellar firing deficiency in a mouse model of spinocerebellar ataxia type 6
4-aminopyridine reverses ataxia and cerebellar firing deficiency in a mouse model of spinocerebellar ataxia type 6 Open
Spinocerebellar ataxia type 6 (SCA6) is a devastating midlife-onset autosomal dominant motor control disease with no known treatment. Using a hyper-expanded polyglutamine (84Q) knock-in mouse, we found that cerebellar Purkinje cell firing …