Mouse Ataxin-2 Expansion Downregulates CamKII and Other Calcium Signaling Factors, Impairing Granule—Purkinje Neuron Synaptic Strength

The RNA-Binding Protein ATXN2 is Expressed during Megakaryopoiesis and May Control Timing of Gene Expression

(2020) Marten Hansen et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

ALS-associated genes in SCA2 mouse spinal cord transcriptomes

(2020) Daniel R Scoles et al.   HUMAN MOLECULAR GENETICS

Mid-Gestation lethality of Atxn2l-Ablated Mice

(2020) Jana Key et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

An update on the Golgi staining technique improving cerebellar cell type specificity

(2019) N. Czechowska et al.   HISTOCHEMISTRY AND CELL BIOLOGY

Activity-Dependent Secretion of Synaptic Organizer Cbln1 from Lysosomes in Granule Cell Axons

(2019) Keiji Ibata et al.   NEURON

Generation of an Atxn2-CAG100 knock-in mouse reveals N-acetylaspartate production deficit due to early Nat8l dysregulation

(2019) Nesli-Ece Sen et al.   NEUROBIOLOGY OF DISEASE

CaM Kinase: Still Inspiring at 40

(2019) K. Ulrich Bayer et al.   NEURON

In Human and Mouse Spino-Cerebellar Tissue, Ataxin-2 Expansion Affects Ceramide-Sphingomyelin Metabolism

(2019) Sen et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Disrupted Calcium Signaling in Animal Models of Human Spinocerebellar Ataxia (SCA)

(2019) Francesca Prestori et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Regulation of actin dynamics during structural plasticity of dendritic spines: Signaling messengers and actin-binding proteins

(2018) Jelena Borovac et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Sensory Processing Phenotypes in Fragile X Syndrome

(2018) Maham Rais et al.   ASN Neuro

CaMKII Isoforms in Learning and Memory: Localization and Function

(2018) Gisela Zalcman et al.   Frontiers in Molecular Neuroscience

Gene co-expression network analysis for identifying modules and functionally enriched pathways in SCA2

(2017) Lance T. Pflieger et al.   HUMAN MOLECULAR GENETICS

Progression of pathology in PINK1-deficient mouse brain from splicing via ubiquitination, ER stress, and mitophagy changes to neuroinflammation

(2017) Sylvia Torres-Odio et al.   Journal of Neuroinflammation

Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice

(2017) Lindsay A. Becker et al.   NATURE

Antisense oligonucleotide therapy for spinocerebellar ataxia type 2

(2017) Daniel R. Scoles et al.   NATURE

Polyglutamine spinocerebellar ataxias — from genes to potential treatments

(2017) Henry L. Paulson et al.   NATURE REVIEWS NEUROSCIENCE

Efficient Prevention of Neurodegenerative Diseases by Depletion of Starvation Response Factor Ataxin-2

(2017) Georg Auburger et al.   TRENDS IN NEUROSCIENCES

Clinical and neuropathological features of ALS/FTD with TIA1 mutations

(2017) Veronica Hirsch-Reinshagen et al.   Acta Neuropathologica Communications

Long-Term Potentiation: From CaMKII to AMPA Receptor Trafficking

(2016) Bruce E. Herring et al.   Annual Review of Physiology

Mammalian ataxin-2 modulates translation control at the pre-initiation complex via PI3K/mTOR and is induced by starvation

(2016) Isabel Lastres-Becker et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

On the distribution of intranuclear and cytoplasmic aggregates in the brainstem of patients with spinocerebellar ataxia type 2 and 3

(2016) Kay Seidel et al.   BRAIN PATHOLOGY

Atxn2 Knockout and CAG42-Knock-in Cerebellum Shows Similarly Dysregulated Expression in Calcium Homeostasis Pathway

(2016) Melanie Vanessa Halbach et al.   CEREBELLUM

Quantitative Global Proteomics of Yeast PBP1 Deletion Mutants and Their Stress Responses Identifies Glucose Metabolism, Mitochondrial, and Stress Granule Changes

(2016) Gunnar Seidel et al.   JOURNAL OF PROTEOME RESEARCH

Ataxin-2 (Atxn2)-Knock-Out Mice Show Branched Chain Amino Acids and Fatty Acids Pathway Alterations

(2016) David Meierhofer et al.   MOLECULAR & CELLULAR PROTEOMICS

Search for SCA2 blood RNA biomarkers highlights Ataxin-2 as strong modifier of the mitochondrial factor PINK1 levels

(2016) Nesli Ece Sen et al.   NEUROBIOLOGY OF DISEASE

Spatio-temporal regulations and functions of neuronal alternative RNA splicing in developing and adult brains

(2016) Takatoshi Iijima et al.   NEUROSCIENCE RESEARCH

An alternative splicing switch shapes neurexin repertoires in principal neurons versus interneurons in the mouse hippocampus

(2016) Thi-Minh Nguyen et al.   eLife

Ca2+ homeostasis and endoplasmic reticulum (ER) stress: An integrated view of calcium signaling

(2015) Joachim Krebs et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

No parkinsonism in SCA2 and SCA3 despite severe neurodegeneration of the dopaminergic substantia nigra

(2015) Ludger Schöls et al.   BRAIN

From Mice to Men: TRPC3 in Cerebellar Ataxia

(2015) Esther B. E. Becker   CEREBELLUM

PAS kinase is activated by direct SNF1-dependent phosphorylation and mediates inhibition of TORC1 through the phosphorylation and activation of Pbp1

(2015) Desiree DeMille et al.   MOLECULAR BIOLOGY OF THE CELL

A cytosolic network suppressing mitochondria-mediated proteostatic stress and cell death

(2015) Xiaowen Wang et al.   NATURE

Panorama of ancient metazoan macromolecular complexes

(2015) Cuihong Wan et al.   NATURE

Deletion of Inpp5a causes ataxia and cerebellar degeneration in mice

(2015) Andy W. Yang et al.   NEUROGENETICS

Genetic ablation of ataxin-2 increases several global translation factors in their transcript abundance but decreases translation rate

(2015) M. Fittschen et al.   NEUROGENETICS

Neuroligins Sculpt Cerebellar Purkinje-Cell Circuits by Differential Control of Distinct Classes of Synapses

(2015) Bo Zhang et al.   NEURON

Dystroglycan Binding to α-Neurexin Competes with Neurexophilin-1 and Neuroligin in the Brain

(2014) Carsten Reissner et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Neuronal cell type–specific alternative splicing is regulated by the KH domain protein SLM1

(2014) Takatoshi Iijima et al.   JOURNAL OF CELL BIOLOGY

Poly-A Binding Protein-1 Localization to a Subset of TDP-43 Inclusions in Amyotrophic Lateral Sclerosis Occurs More Frequently in Patients Harboring an Expansion inC9orf72

(2014) Leeanne McGurk et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Progression of early features of spinocerebellar ataxia type 2 in individuals at risk: a longitudinal study

(2014) Luis Velázquez-Pérez et al.   LANCET NEUROLOGY

Cartography of neurexin alternative splicing mapped by single-molecule long-read mRNA sequencing

(2014) Barbara Treutlein et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Ataxin-2 Modulates the Levels of Grb2 and Src but Not Ras Signaling

(2013) Jessica Drost et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Involvement of the cholinergic basal forebrain nuclei in spinocerebellar ataxia type 2 (SCA2)

(2013) U. Rüb et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

FMRP and Ataxin-2 function together in long-term olfactory habituation and neuronal translational control

(2013) I. P. Sudhakaran et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

RNA-binding protein Sam68 controls synapse number and local  -actin mRNA metabolism in dendrites

(2013) M. E. Klein et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Brain pathology of spinocerebellar ataxias

(2012) Kay Seidel et al.   ACTA NEUROPATHOLOGICA

Distinct TDP-43 pathology in ALS patients with ataxin 2 intermediate-length polyQ expansions

(2012) Michael P. Hart et al.   ACTA NEUROPATHOLOGICA

Estimation of survival in Spinocerebellar Ataxia type 2 Cuban patients

(2012) LE Almaguer-Mederos et al.   CLINICAL GENETICS

Ataxin-2 interacts with FUS and intermediate-length polyglutamine expansions enhance FUS-related pathology in amyotrophic lateral sclerosis

(2012) Manal A. Farg et al.   HUMAN MOLECULAR GENETICS

Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2

(2012) S. T. Hansen et al.   HUMAN MOLECULAR GENETICS

Chronic Suppression of Inositol 1,4,5-Triphosphate Receptor-Mediated Calcium Signaling in Cerebellar Purkinje Cells Alleviates Pathological Phenotype in Spinocerebellar Ataxia 2 Mice

(2012) A. W. Kasumu et al.   JOURNAL OF NEUROSCIENCE

ALS-Associated Ataxin 2 PolyQ Expansions Enhance Stress-Induced Caspase 3 Activation and Increase TDP-43 Pathological Modifications

(2012) M. P. Hart et al.   JOURNAL OF NEUROSCIENCE

Transient Sequestration of TORC1 into Stress Granules during Heat Stress

(2012) Terunao Takahara et al.   MOLECULAR CELL

ATXN2-CAG42 Sequesters PABPC1 into Insolubility and Induces FBXW8 in Cerebellum of Old Ataxic Knock-In Mice

(2012) Ewa Damrath et al.   PLoS Genetics

SAM68 Regulates Neuronal Activity-Dependent Alternative Splicing of Neurexin-1

(2011) Takatoshi Iijima et al.   CELL

Ataxin-2 repeat-length variation and neurodegeneration

(2011) O. A. Ross et al.   HUMAN MOLECULAR GENETICS

Activity-dependent regulation of synaptic strength by PSD-95 in CA1 neurons

(2011) Peng Zhang et al.   JOURNAL OF NEUROPHYSIOLOGY

Impaired Locomotor Learning and Altered Cerebellar Synaptic Plasticity in pep-19/pcp4-Null Mice

(2011) P. Wei et al.   MOLECULAR AND CELLULAR BIOLOGY

Excitation-induced ataxin-3 aggregation in neurons from patients with Machado–Joseph disease

(2011) Philipp Koch et al.   NATURE

The modulation of Amyotrophic Lateral Sclerosis risk by Ataxin-2 intermediate polyglutamine expansions is a specific effect

(2011) Suzana Gispert et al.   NEUROBIOLOGY OF DISEASE

The Ataxin-2 protein is required for microRNA function and synapse-specific long-term olfactory habituation

(2011) C. McCann et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Spinocerebellar Ataxia Type 2 (SCA2): Identification of Early Brain Degeneration in One Monozygous Twin in the Initial Disease Stage

(2010) Franziska Hoche et al.   CEREBELLUM

Functional Integration of Calcium Regulatory Mechanisms at Purkinje Neuron Synapses

(2010) Ruth M. Empson et al.   CEREBELLUM

Sam68 sequestration and partial loss of function are associated with splicing alterations in FXTAS patients

(2010) Chantal Sellier et al.   EMBO JOURNAL

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

(2010) Andrew C. Elden et al.   NATURE

Disruption of LGI1–linked synaptic complex causes abnormal synaptic transmission and epilepsy

(2010) Yuko Fukata et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Saccade velocity is reduced in presymptomatic spinocerebellar ataxia type 2

(2009) L. Velázquez-Pérez et al.   CLINICAL NEUROPHYSIOLOGY

Deranged Calcium Signaling and Neurodegeneration in Spinocerebellar Ataxia Type 2

(2009) J. Liu et al.   JOURNAL OF NEUROSCIENCE

Ca2+/Calmodulin-dependent Protein Kinases

(2008) M. T. Swulius et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Ataxin-2 associates with the endocytosis complex and affects EGF receptor trafficking

(2008) David Nonis et al.   CELLULAR SIGNALLING

Ataxin-2 associates with rough endoplasmic reticulum

(2008) Simone van de Loo et al.   EXPERIMENTAL NEUROLOGY

Insulin receptor and lipid metabolism pathology in ataxin-2 knock-out mice

(2008) Isabel Lastres-Becker et al.   HUMAN MOLECULAR GENETICS