Albert R. La Spada Department of Neurology Duke University School of Medicine Biography Publications Institution JoVE Articles Albert R. La Spada has not added a biography. If you are Albert R. La Spada and would like to personalize this page please email our Author Liaison for assistance. Publications Mutant Huntingtin Impairs PNKP and ATXN3, Disrupting DNA Repair and Transcription ELife. Apr, 2019 | Pubmed ID: 30994454 Gene Expression Analysis Reveals Early Dysregulation of Disease Pathways and Links Chmp7 to Pathogenesis of Spinal and Bulbar Muscular Atrophy Scientific Reports. Mar, 2019 | Pubmed ID: 30837566 Astroglial-targeted Expression of the Fragile X CGG Repeat Premutation in Mice Yields RAN Translation, Motor Deficits and Possible Evidence for Cell-to-cell Propagation of FXTAS Pathology Acta Neuropathologica Communications. Feb, 2019 | Pubmed ID: 30808398 Metabolic and Organelle Morphology Defects in Mice and Human Patients Define Spinocerebellar Ataxia Type 7 As a Mitochondrial Disease Cell Reports. Jan, 2019 | Pubmed ID: 30699348 CCP1 Promotes Mitochondrial Fusion and Motility to Prevent Purkinje Cell Neuron Loss in Mice The Journal of Cell Biology. Jan, 2019 | Pubmed ID: 30337352 TFEB Dysregulation As a Driver of Autophagy Dysfunction in Neurodegenerative Disease: Molecular Mechanisms, Cellular Processes, and Emerging Therapeutic Opportunities Neurobiology of Disease. 02, 2019 | Pubmed ID: 29852219 Antisense Oligonucleotides Targeting Mutant Ataxin-7 Restore Visual Function in a Mouse Model of Spinocerebellar Ataxia Type 7 Science Translational Medicine. Oct, 2018 | Pubmed ID: 30381411 Converging Pathways in Neurodegeneration, from Genetics to Mechanisms Nature Neuroscience. 10, 2018 | Pubmed ID: 30258237 Senataxin, A Novel Helicase at the Interface of RNA Transcriptome Regulation and Neurobiology: From Normal Function to Pathological Roles in Motor Neuron Disease and Cerebellar Degeneration Advances in Neurobiology. 2018 | Pubmed ID: 29916023 Fasting Activates Fatty Acid Oxidation to Enhance Intestinal Stem Cell Function During Homeostasis and Aging Cell Stem Cell. 05, 2018 | Pubmed ID: 29727683 Senataxin Mutations Elicit Motor Neuron Degeneration Phenotypes and Yield TDP-43 Mislocalization in ALS4 Mice and Human Patients Acta Neuropathologica. 09, 2018 | Pubmed ID: 29725819 MAP4K3 Mediates Amino Acid-dependent Regulation of Autophagy Via Phosphorylation of TFEB Nature Communications. 03, 2018 | Pubmed ID: 29507340 신경 근육 질환의 마우스 모델의 행동 현상형에 대한 저렴한 걸음걸이 분석 Virginia Wertman1,2, Anastasia Gromova1,2,3, Albert R. La Spada1,2,4,5, Constanza J. Cortes1,2 1Department of Neurology, Duke University School of Medicine, 2Duke Center for Neurodegeneration & Neurotherapeutics, Duke University School of Medicine, 3Biomedical Sciences Graduate Program, University of California San Diego, 4Department of Neurobiology, Duke University School of Medicine, 5Department of Cell Biology, Duke University School of Medicine JoVE 59878 Behavior
신경 근육 질환의 마우스 모델의 행동 현상형에 대한 저렴한 걸음걸이 분석 Virginia Wertman1,2, Anastasia Gromova1,2,3, Albert R. La Spada1,2,4,5, Constanza J. Cortes1,2 1Department of Neurology, Duke University School of Medicine, 2Duke Center for Neurodegeneration & Neurotherapeutics, Duke University School of Medicine, 3Biomedical Sciences Graduate Program, University of California San Diego, 4Department of Neurobiology, Duke University School of Medicine, 5Department of Cell Biology, Duke University School of Medicine JoVE 59878 Behavior
