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Full-Text Articles in Biochemistry, Biophysics, and Structural Biology
Expression Of Fused In Sarcoma Mutations In Mice Recapitulates The Neuropathology Of Fus Proteinopathies And Provides Insight Into Disease Pathogenesis, Christophe Verbeeck, Mariely Dejesus-Hernandez, Carolina Ceballos-Diaz, Jannet Kocerha, Todd Golde, Pritam Das, Rosa Rademakers, Dennis W. Dickson, Thomas Kukar
Expression Of Fused In Sarcoma Mutations In Mice Recapitulates The Neuropathology Of Fus Proteinopathies And Provides Insight Into Disease Pathogenesis, Christophe Verbeeck, Mariely Dejesus-Hernandez, Carolina Ceballos-Diaz, Jannet Kocerha, Todd Golde, Pritam Das, Rosa Rademakers, Dennis W. Dickson, Thomas Kukar
Jannet Kocerha
Background: Mutations in the gene encoding the RNA-binding protein fused in sarcoma (FUS) can cause familial and sporadic amyotrophic lateral sclerosis (ALS) and rarely frontotemproal dementia (FTD). FUS accumulates in neuronal cytoplasmic inclusions (NCIs) in ALS patients with FUS mutations. FUS is also a major pathologic marker for a group of less common forms of frontotemporal lobar degeneration (FTLD), which includes atypical FTLD with ubiquitinated inclusions (aFTLD-U), neuronal intermediate filament inclusion disease (NIFID) and basophilic inclusion body disease (BIBD). These diseases are now called FUS proteinopathies, because they share this disease marker. It is unknown how FUS mutations cause disease …
The Path To Microrna Therapeutics In Psychiatric And Neurodegenerative Disorders, Anthony W. S. Chan, Jannet Kocerha
The Path To Microrna Therapeutics In Psychiatric And Neurodegenerative Disorders, Anthony W. S. Chan, Jannet Kocerha
Jannet Kocerha
The microRNA (miRNA) class of non-coding RNAs exhibit a diverse range of regulatory roles in neuronal functions that are conserved from lower vertebrates to primates. Disruption of miRNA expression has compellingly been linked to pathogenesis in neuropsychiatric and neurodegenerative disorders, such as schizophrenia, Alzheimer’s disease, and autism. The list of transcript targets governed by a single miRNA provide a molecular paradigm applicable for therapeutic intervention. Indeed, reports have shown that specific manipulation of a miRNA in cell or animal models can significantly alter phenotypes linked with neurological disease. Here, we review how a diverse range of biological systems, including Drosophila, …