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

Molecular & cellular proteomics, 2016-05, Vol.15 (5), p.1728-1739

2016

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Translation Fidelity and Respiration Deficits in CLPP-Deficient Tissues: Mechanistic Insights from Mitochondrial Complexome Profiling

International journal of molecular sciences, 2023-12, Vol.24 (24), p.17503

2023

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Loss of mitochondrial protease ClpP protects mice from diet‐induced obesity and insulin resistance

EMBO reports, 2018-03, Vol.19 (3), p.n/a

2018

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Increased presence of nuclear DNAJA3 and upregulation of cytosolic STAT1 and of nucleic acid sensors trigger innate immunity in the ClpP-null mouse

Neurogenetics, 2021-10, Vol.22 (4), p.297-312

2021

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The Bacterial ClpXP-ClpB Family Is Enriched with RNA-Binding Protein Complexes

Cells (Basel, Switzerland), 2022-08, Vol.11 (15), p.2370

2022

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

Cerebellum (London, England), 2017-02, Vol.16 (1), p.68-81

2017

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Mid-Gestation lethality of Atxn2l -Ablated Mice

International journal of molecular sciences, 2020-07, Vol.21 (14), p.5124

2020

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Atxn2-CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppression

Neurobiology of disease, 2021-05, Vol.152, p.105289-105289, Article 105289

2021

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

Journal of neuroinflammation, 2017-08, Vol.14 (1), p.154-154, Article 154

2017

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SerThr-PhosphoProteome of Brain from Aged PINK1-KO+A53T-SNCA Mice Reveals pT1928-MAP1B and pS3781-ANK2 Deficits, as Hub between Autophagy and Synapse Changes

International journal of molecular sciences, 2019-07, Vol.20 (13), p.3284

2019

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

International journal of molecular sciences, 2019-11, Vol.20 (23), p.5854

2019

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

Neurobiology of disease, 2019-12, Vol.132, p.104559-104559, Article 104559

2019

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Primary Skin Fibroblasts as a Model of Parkinson's Disease

Molecular neurobiology, 2012-08, Vol.46 (1), p.20-27

2012

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Pathomechanisms and compensatory efforts related to Parkinsonian speech

NeuroImage clinical, 2014-01, Vol.4 (C), p.82-97

2014

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CLPP-Null Eukaryotes with Excess Heme Biosynthesis Show Reduced L-arginine Levels, Probably via CLPX-Mediated OAT Activation

Biomolecules (Basel, Switzerland), 2024-02, Vol.14 (2), p.241

2024

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Impaired Photic Entrainment of Spontaneous Locomotor Activity in Mice Overexpressing Human Mutant α-Synuclein

International journal of molecular sciences, 2018-06, Vol.19 (6), p.1651

2018

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

International journal of molecular sciences, 2020-09, Vol.21 (18), p.6673

2020

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ATXN2-CAG42 sequesters PABPC1 into insolubility and induces FBXW8 in cerebellum of old ataxic knock-in mice

PLoS genetics, 2012-08, Vol.8 (8), p.e1002920

2012

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

Neurobiology of disease, 2016-12, Vol.96, p.115-126

2016

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A53T-alpha-synuclein overexpression impairs dopamine signaling and striatal synaptic plasticity in old mice

PloS one, 2010-07, Vol.5 (7), p.e11464-e11464

2010

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Loss of PINK1 impairs stress-induced autophagy and cell survival

PloS one, 2014-04, Vol.9 (4), p.e95288-e95288

2014

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Genetic mutations linked to Parkinson's disease differentially control nucleolar activity in pre-symptomatic mouse models

Disease models & mechanisms, 2017-05, Vol.10 (5), p.633-643

2017

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

Neurobiology of disease, 2012-01, Vol.45 (1), p.356-361

2012

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Methyl-Arginine Profile of Brain from Aged PINK1-KO+A53T-SNCA Mice Suggests Altered Mitochondrial Biogenesis

Parkinson's disease, 2016-01, Vol.2016, p.4686185-13

2016

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Mitochondrial dysfunction, peroxidation damage and changes in glutathione metabolism in PARK6

Neurobiology of disease, 2007-02, Vol.25 (2), p.401-411

2007

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

Translation Fidelity and Respiration Deficits in CLPP-Deficient Tissues: Mechanistic Insights from Mitochondrial Complexome Profiling

Loss of mitochondrial protease ClpP protects mice from diet‐induced obesity and insulin resistance

Increased presence of nuclear DNAJA3 and upregulation of cytosolic STAT1 and of nucleic acid sensors trigger innate immunity in the ClpP-null mouse

The Bacterial ClpXP-ClpB Family Is Enriched with RNA-Binding Protein Complexes

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

Mid-Gestation lethality of Atxn2l -Ablated Mice

Atxn2-CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppression

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

SerThr-PhosphoProteome of Brain from Aged PINK1-KO+A53T-SNCA Mice Reveals pT1928-MAP1B and pS3781-ANK2 Deficits, as Hub between Autophagy and Synapse Changes

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

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

Primary Skin Fibroblasts as a Model of Parkinson's Disease

Pathomechanisms and compensatory efforts related to Parkinsonian speech

CLPP-Null Eukaryotes with Excess Heme Biosynthesis Show Reduced L-arginine Levels, Probably via CLPX-Mediated OAT Activation

Impaired Photic Entrainment of Spontaneous Locomotor Activity in Mice Overexpressing Human Mutant α-Synuclein

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

ATXN2-CAG42 sequesters PABPC1 into insolubility and induces FBXW8 in cerebellum of old ataxic knock-in mice

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

A53T-alpha-synuclein overexpression impairs dopamine signaling and striatal synaptic plasticity in old mice

Loss of PINK1 impairs stress-induced autophagy and cell survival

Genetic mutations linked to Parkinson's disease differentially control nucleolar activity in pre-symptomatic mouse models

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

Methyl-Arginine Profile of Brain from Aged PINK1-KO+A53T-SNCA Mice Suggests Altered Mitochondrial Biogenesis

Mitochondrial dysfunction, peroxidation damage and changes in glutathione metabolism in PARK6

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