Warunee Dansithong
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View article: Targeting STAU1 prevents p53 apoptotic signaling in neurodegeneration
Targeting STAU1 prevents p53 apoptotic signaling in neurodegeneration Open
View article: A human Staufen1 BAC transgenic mouse exhibits abnormal autophagy and neurodegeneration across the central nervous system
A human Staufen1 BAC transgenic mouse exhibits abnormal autophagy and neurodegeneration across the central nervous system Open
RNA-binding proteins (RBPs) play an essential role in development, normal functioning and human disease. Staufen1 (STAU1) is an RBP that regulates mRNA degradation and subcellular localization, and is part of the ATXN2 protein complex. Pre…
View article: Staufen2 dysregulation in neurodegenerative disease
Staufen2 dysregulation in neurodegenerative disease Open
Staufen2 (STAU2) is an RNA-binding protein that controls mRNA trafficking and expression. Previously, we showed that its paralog, Staufen1 (STAU1), was overabundant in cellular and mouse models of neurodegenerative diseases and amyotrophic…
View article: Viral vector-mediated <i>SLC9A6</i> gene replacement reduces cerebellar dysfunction in the <i>shaker</i> rat model of Christianson syndrome
Viral vector-mediated <i>SLC9A6</i> gene replacement reduces cerebellar dysfunction in the <i>shaker</i> rat model of Christianson syndrome Open
Christianson syndrome (CS) is an x-linked recessive neurodevelopmental and neurodegenerative condition characterized by severe intellectual disability, cerebellar degeneration, ataxia, and epilepsy. Mutations to the SLC9A6 gene are respons…
View article: Cerebellar Micro-RNA Profile in a Mouse Model of Spinocerebellar Ataxia Type 2
Cerebellar Micro-RNA Profile in a Mouse Model of Spinocerebellar Ataxia Type 2 Open
Our study establishes the role of both DEmiRs and their targets in SCA2 pathogenesis. By expressing mutant ATXN2 under the control of its endogenous regulatory elements in the SCA2-BAC-ATXN2[Q72] mouse model, we identified a …
View article: Slc9a6 mutation causes Purkinje cell loss and ataxia in the <i>shaker</i> rat
Slc9a6 mutation causes Purkinje cell loss and ataxia in the <i>shaker</i> rat Open
The shaker rat carries a naturally occurring mutation leading to progressive ataxia characterized by Purkinje cell (PC) loss. We previously reported on fine-mapping the shaker locus to the long arm of the rat X chromosome. In this work, we…
View article: Targeting Staufen 1 with antisense oligonucleotides for treating ALS and SCA2
Targeting Staufen 1 with antisense oligonucleotides for treating ALS and SCA2 Open
Staufen1 (STAU1) is a multifunctional RNA binding protein that controls mRNA degradation and subcellular localization. STAU1 interacts with the ATXN2 protein, that is polyglutamine expanded in spinocerebellar ataxia type 2 (SCA2). We previ…
View article: Staufen Impairs Autophagy in Neurodegeneration
Staufen Impairs Autophagy in Neurodegeneration Open
Objective The mechanistic target of rapamycin (mTOR) kinase is one of the master coordinators of cellular stress responses, regulating metabolism, autophagy, and apoptosis. We recently reported that staufen1 (STAU1), a stress granule (SG) …
View article: A quantitative high-throughput screen identifies compounds that lower expression of the SCA2-and ALS-associated gene ATXN2
A quantitative high-throughput screen identifies compounds that lower expression of the SCA2-and ALS-associated gene ATXN2 Open
View article: Slc9a6 mutation causes Purkinje cell loss and ataxia in the <i>shaker</i> rat
Slc9a6 mutation causes Purkinje cell loss and ataxia in the <i>shaker</i> rat Open
Background The shaker rat carries a naturally occurring mutation leading to progressive ataxia characterized by Purkinje cell (PC) loss. We previously reported on fine-mapping the shaker locus to the long arm of the rat X chromosome. In th…
View article: The AKT modulator A-443654 reduces α-synuclein expression and normalizes ER stress and autophagy
The AKT modulator A-443654 reduces α-synuclein expression and normalizes ER stress and autophagy Open
Accumulation of α-synuclein is a main underlying pathological feature of Parkinson's disease and α-synucleinopathies, for which lowering expression of the α-synuclein gene (SNCA) is a potential therapeutic avenue. Using a cell-based lucife…
View article: Correction: Generation of SNCA Cell Models Using Zinc Finger Nuclease (ZFN) Technology for Efficient High-Throughput Drug Screening
Correction: Generation of SNCA Cell Models Using Zinc Finger Nuclease (ZFN) Technology for Efficient High-Throughput Drug Screening Open
[This corrects the article DOI: 10.1371/journal.pone.0136930.].
View article: Staufen1 in Human Neurodegeneration
Staufen1 in Human Neurodegeneration Open
Objective Mutations in the ATXN2 gene (CAG expansions ≥32 repeats) can be a rare cause of Parkinson's disease and amyotrophic lateral sclerosis (ALS). We recently reported that the stress granule (SG) protein Staufen1 (STAU1) was overabund…
View article: Correction: Staufen 1 amplifies proapoptotic activation of the unfolded protein response
Correction: Staufen 1 amplifies proapoptotic activation of the unfolded protein response Open
View article: Toward newborn screening of metachromatic leukodystrophy: results from analysis of over 27,000 newborn dried blood spots
Toward newborn screening of metachromatic leukodystrophy: results from analysis of over 27,000 newborn dried blood spots Open
View article: Altered Capicua expression drives regional Purkinje neuron vulnerability through ion channel gene dysregulation in spinocerebellar ataxia type 1
Altered Capicua expression drives regional Purkinje neuron vulnerability through ion channel gene dysregulation in spinocerebellar ataxia type 1 Open
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…
View article: Staufen 1 amplifies proapoptotic activation of the unfolded protein response
Staufen 1 amplifies proapoptotic activation of the unfolded protein response Open
Staufen-1 (STAU1) is an RNA-binding protein that becomes highly overabundant in numerous neurodegenerative disease models, including those carrying mutations in presenilin1 (PSEN1), microtubule-associated protein tau ( MAPT ), huntingtin (…
View article: ALS-associated genes in SCA2 mouse spinal cord transcriptomes
ALS-associated genes in SCA2 mouse spinal cord transcriptomes Open
The spinocerebellar ataxia type 2 (SCA2) gene ATXN2 has a prominent role in the pathogenesis and treatment of amyotrophic lateral sclerosis (ALS). In addition to cerebellar ataxia, motor neuron disease is often seen in SCA2, and ATXN2 CAG …
View article: Staufen 1 amplifies pro-apoptotic activation of the unfolded protein response
Staufen 1 amplifies pro-apoptotic activation of the unfolded protein response Open
Staufen-1 (STAU1) is an RNA binding protein that becomes highly overabundant in numerous neurodegenerative disease models, including those carrying mutations in presenilin1 (PSEN1), microtubule associated protein tau ( MAPT ), huntingtin (…
View article: Staufen blocks autophagy in neurodegeneration
Staufen blocks autophagy in neurodegeneration Open
Objective The mechanistic target of rapamycin (mTOR) kinase is one of the master coordinators of cellular stress responses, regulating metabolism, autophagy, and apoptosis. We recently reported that Staufen1 (STAU1), a stress granule (SG) …
View article: Staufen1 links RNA stress granules and autophagy in a model of neurodegeneration
Staufen1 links RNA stress granules and autophagy in a model of neurodegeneration Open
Spinocerebellar ataxia type 2 (SCA2) is a neurodegenerative disease caused by expansion of polyglutamine tract in the ATXN2 protein. We identified Staufen1 (STAU1) as an interactor of ATXN2, and showed elevation in cells from SCA2 patients…
View article: Gene co-expression network analysis for identifying modules and functionally enriched pathways in SCA2
Gene co-expression network analysis for identifying modules and functionally enriched pathways in SCA2 Open
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease caused by CAG repeat expansion in the ATXN2 gene. The repeat resides in an encoded region of the gene resulting in polyglutamine (polyQ) expansion whic…
View article: Muscleblind-like 3 deficit results in a spectrum of age-associated pathologies observed in myotonic dystrophy
Muscleblind-like 3 deficit results in a spectrum of age-associated pathologies observed in myotonic dystrophy Open
Myotonic dystrophy type I (DM1) exhibits distinctive disease specific phenotypes and the accelerated onset of a spectrum of age-associated pathologies. In DM1, dominant effects of expanded CUG repeats result in part from the inactivation o…
View article: Spontaneous <i>shaker</i> rat mutant – a new model for X-linked tremor/ataxia
Spontaneous <i>shaker</i> rat mutant – a new model for X-linked tremor/ataxia Open
The shaker rat is an X-linked recessive spontaneous model of progressive Purkinje cell (PC) degeneration exhibiting a shaking ataxia and wide stance. Generation of Wistar Furth (WF)/Brown Norwegian (BN) F1 hybrids and genetic mapping of F2…
View article: Co-expression networks in generation of induced pluripotent stem cells
Co-expression networks in generation of induced pluripotent stem cells Open
We developed an adenoviral vector, in which Yamanaka's four reprogramming factors (RFs) were controlled by individual CMV promoters in a single cassette (Ad-SOcMK). This permitted coordinated expression of RFs (SOX2, OCT3/4, c-MYC and KLF4…
View article: Generation of SNCA Cell Models Using Zinc Finger Nuclease (ZFN) Technology for Efficient High-Throughput Drug Screening
Generation of SNCA Cell Models Using Zinc Finger Nuclease (ZFN) Technology for Efficient High-Throughput Drug Screening Open
[This corrects the article DOI: 10.1371/journal.pone.0136930.].
View article: Muscleblind-Like 1 and Muscleblind-Like 3 Depletion Synergistically Enhances Myotonia by Altering Clc-1 RNA Translation
Muscleblind-Like 1 and Muscleblind-Like 3 Depletion Synergistically Enhances Myotonia by Altering Clc-1 RNA Translation Open
View article: Repeat Associated Non-AUG Translation (RAN Translation) Dependent on Sequence Downstream of the ATXN2 CAG Repeat
Repeat Associated Non-AUG Translation (RAN Translation) Dependent on Sequence Downstream of the ATXN2 CAG Repeat Open
Spinocerebellar ataxia type 2 (SCA2) is a progressive autosomal dominant disorder caused by the expansion of a CAG tract in the ATXN2 gene. The SCA2 disease phenotype is characterized by cerebellar atrophy, gait ataxia, and slow saccades. …
View article: Ataxin-2 Regulates RGS8 Translation in a New BAC-SCA2 Transgenic Mouse Model
Ataxin-2 Regulates RGS8 Translation in a New BAC-SCA2 Transgenic Mouse Model Open
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant disorder with progressive degeneration of cerebellar Purkinje cells (PCs) and other neurons caused by expansion of a glutamine (Q) tract in the ATXN2 protein. We generated BAC t…