Altered Levels of Proteins and Phosphoproteins, in the Absence of Early Causative Transcriptional Changes, Shape the Molecular Pathogenesis in the Brain of Young Presymptomatic Ki91 SCA3/MJD Mouse

• Published in: Molecular neurobiology Vol. 56; no. 12; pp. 8168 - 8202
• Main Authors: Wiatr, Kalina, Piasecki, Piotr, Marczak, Łukasz, Wojciechowski, Paweł, Kurkowiak, Małgorzata, Płoski, Rafał, Rydzanicz, Małgorzata, Handschuh, Luiza, Jungverdorben, Johannes, Brüstle, Oliver, Figlerowicz, Marek, Figiel, Maciej
• Format: Journal Article
• Language: English
• Published: New York Springer US 01.12.2019; Springer Nature B.V
• Subjects: Age Factors; Animals; Ataxin; Ataxin-3 - genetics; Ataxin-3 - metabolism; Axons; Biomedical and Life Sciences; Biomedicine; Brain; Brain - metabolism; Brain - pathology; Casein; Cell Biology; Cells, Cultured; Cerebellum; Cytoskeleton; Deoxyribonucleic acid; DNA; DNA damage; Humans; Kinases; Machado-Joseph disease; Machado-Joseph Disease - genetics; Machado-Joseph Disease - metabolism; Mice; Mice, 129 Strain; Mice, Inbred C57BL; Mice, Transgenic; Mitochondria; Nerve Tissue Proteins - genetics; Nerve Tissue Proteins - metabolism; Neurobiology; Neurodegeneration; Neurodegenerative diseases; Neurology; Neurosciences; Pathogenesis; Phosphoproteins; Phosphoproteins - genetics; Phosphoproteins - metabolism; Phosphorylation; Polyglutamine diseases; Protein kinase A; Proteins; Proteomics; Tau protein; Transcription; Transcription, Genetic - physiology; Translation termination; Trinucleotide repeat diseases; Spinocerebellar; Presymptomatic; Ataxin-3; Mouse; SCA3; MJD; CAG; Ataxia; Proteome; Phosphoproteome; Knock-in; PolyQ

Spinocerebellar ataxia type 3 (SCA3/MJD) is a polyQ neurodegenerative disease where the presymptomatic phase of pathogenesis is unknown. Therefore, we investigated the molecular network of transcriptomic and proteomic triggers in young presymptomatic SCA3/MJD brain from Ki91 knock-in mouse. We found...