Albert R. La Spada Division of Genetics, Departments of Pediatrics and Cellular and Molecular Medicine, and the Institute for Genomic Medicine University of California, San Diego - Rady Children’s Hospital 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 Finding a Sirtuin Truth in Huntington's Disease Nature Medicine. Jan, 2012 | Pubmed ID: 22227661 Ataxin-7 Associates with Microtubules and Stabilizes the Cytoskeletal Network Human Molecular Genetics. Mar, 2012 | Pubmed ID: 22100762 PGC-1α at the Intersection of Bioenergetics Regulation and Neuron Function: From Huntington's Disease to Parkinson's Disease and Beyond Progress in Neurobiology. Nov, 2011 | Pubmed ID: 22100502 Spinocerebellar Ataxia Type 7 Cerebellar Disease Requires the Coordinated Action of Mutant Ataxin-7 in Neurons and Glia, and Displays Non-cell-autonomous Bergmann Glia Degeneration The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Nov, 2011 | Pubmed ID: 22072678 CTCF Regulates Ataxin-7 Expression Through Promotion of a Convergently Transcribed, Antisense Noncoding RNA Neuron. Jun, 2011 | Pubmed ID: 21689595 An Antisense CAG Repeat Transcript at JPH3 Locus Mediates Expanded Polyglutamine Protein Toxicity in Huntington's Disease-like 2 Mice Neuron. May, 2011 | Pubmed ID: 21555070 Absence of Disturbed Axonal Transport in Spinal and Bulbar Muscular Atrophy Human Molecular Genetics. May, 2011 | Pubmed ID: 21317158 Mitochondrial Autophagy in Neural Function, Neurodegenerative Disease, Neuron Cell Death, and Aging Neurobiology of Disease. Jul, 2011 | Pubmed ID: 20887789 Déjà Vu with a Twist: Transglutaminases in Bioenergetics and Transcriptional Dysfunction in Huntington's Disease EMBO Molecular Medicine. Sep, 2010 | Pubmed ID: 20730854 Proteomics of Dense Core Secretory Vesicles Reveal Distinct Protein Categories for Secretion of Neuroeffectors for Cell-cell Communication Journal of Proteome Research. Oct, 2010 | Pubmed ID: 20695487 Mitochondrial Dysfunction in NnaD Mutant Flies and Purkinje Cell Degeneration Mice Reveals a Role for Nna Proteins in Neuronal Bioenergetics Neuron. Jun, 2010 | Pubmed ID: 20620870 Repeat Expansion Disease: Progress and Puzzles in Disease Pathogenesis Nature Reviews. Genetics. Apr, 2010 | Pubmed ID: 20177426 Memantine Strikes the Perfect Balance Nature Medicine. Dec, 2009 | Pubmed ID: 19966768 Posttranslational Modification of Ataxin-7 at Lysine 257 Prevents Autophagy-mediated Turnover of an N-terminal Caspase-7 Cleavage Fragment The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Dec, 2009 | Pubmed ID: 19955365 ALS Motor Phenotype Heterogeneity, Focality, and Spread: Deconstructing Motor Neuron Degeneration Neurology. Sep, 2009 | Pubmed ID: 19738176 Autophagy Activation and Enhanced Mitophagy Characterize the Purkinje Cells of Pcd Mice Prior to Neuronal Death Molecular Brain. 2009 | Pubmed ID: 19640278 Development of Selective Nutrient Deprivation As a System to Study Autophagy Induction and Regulation in Neurons Autophagy. May, 2009 | Pubmed ID: 19363305 Getting a Handle on Huntington's Disease: Silencing Neurodegeneration Nature Medicine. Mar, 2009 | Pubmed ID: 19265826 Polyglutamine-expanded Androgen Receptor Truncation Fragments Activate a Bax-dependent Apoptotic Cascade Mediated by DP5/Hrk The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Feb, 2009 | Pubmed ID: 19228953 Nutrient Deprivation Induces Neuronal Autophagy and Implicates Reduced Insulin Signaling in Neuroprotective Autophagy Activation The Journal of Biological Chemistry. Jan, 2009 | Pubmed ID: 19017649 CTCF Cis-regulates Trinucleotide Repeat Instability in an Epigenetic Manner: a Novel Basis for Mutational Hot Spot Determination PLoS Genetics. Nov, 2008 | Pubmed ID: 19008940 Androgen Receptor Function in Motor Neuron Survival and Degeneration Physical Medicine and Rehabilitation Clinics of North America. Aug, 2008 | Pubmed ID: 18625411 The Zinc-binding Domain of Nna1 is Required to Prevent Retinal Photoreceptor Loss and Cerebellar Ataxia in Purkinje Cell Degeneration (pcd) Mice Vision Research. Sep, 2008 | Pubmed ID: 18602413 Molecular Pathogenesis and Cellular Pathology of Spinocerebellar Ataxia Type 7 Neurodegeneration Cerebellum (London, England). 2008 | Pubmed ID: 18418675 Visceral Neuropathy and Intestinal Pseudo-obstruction in a Murine Model of a Nuclear Inclusion Disease Gastroenterology. Dec, 2007 | Pubmed ID: 18054568 Proteolytic Cleavage of Ataxin-7 by Caspase-7 Modulates Cellular Toxicity and Transcriptional Dysregulation The Journal of Biological Chemistry. Oct, 2007 | Pubmed ID: 17646170 Akt Blocks Ligand Binding and Protects Against Expanded Polyglutamine Androgen Receptor Toxicity Human Molecular Genetics. Jul, 2007 | Pubmed ID: 17470458 Neurodegeneration: a Case of Arrested Development? Cell. Nov, 2006 | Pubmed ID: 17110325 Thermoregulatory and Metabolic Defects in Huntington's Disease Transgenic Mice Implicate PGC-1alpha in Huntington's Disease Neurodegeneration Cell Metabolism. Nov, 2006 | Pubmed ID: 17055784 Beta-synuclein Modulates Alpha-synuclein Neurotoxicity by Reducing Alpha-synuclein Protein Expression Human Molecular Genetics. Oct, 2006 | Pubmed ID: 16959793 Bergmann Glia Expression of Polyglutamine-expanded Ataxin-7 Produces Neurodegeneration by Impairing Glutamate Transport Nature Neuroscience. Oct, 2006 | Pubmed ID: 16936724 Targeting Protein Aggregation in Neurodegeneration--lessons from Polyglutamine Disorders Expert Opinion on Therapeutic Targets. Aug, 2006 | Pubmed ID: 16848688 Loss of Endogenous Androgen Receptor Protein Accelerates Motor Neuron Degeneration and Accentuates Androgen Insensitivity in a Mouse Model of X-linked Spinal and Bulbar Muscular Atrophy Human Molecular Genetics. Jul, 2006 | Pubmed ID: 16772330 Efficient Recombination-based Methods for Bacterial Artificial Chromosome Fusion and Mutagenesis Gene. Apr, 2006 | Pubmed ID: 16487669 The Purkinje Cell Degeneration 5J Mutation is a Single Amino Acid Insertion That Destabilizes Nna1 Protein Mammalian Genome : Official Journal of the International Mammalian Genome Society. Feb, 2006 | Pubmed ID: 16465590 Ataxin-7 Can Export from the Nucleus Via a Conserved Exportin-dependent Signal The Journal of Biological Chemistry. Feb, 2006 | Pubmed ID: 16314424 Silencing Polyglutamine Degeneration with RNAi Neuron. Dec, 2005 | Pubmed ID: 16337909 Huntington's Disease and Neurogenesis: FGF-2 to the Rescue? Proceedings of the National Academy of Sciences of the United States of America. Dec, 2005 | Pubmed ID: 16330780 Targeting Toxic Proteins for Turnover Nature Medicine. Oct, 2005 | Pubmed ID: 16211038 The Power of the Dark Side: Huntington's Disease Protein and P53 Form a Deadly Alliance Neuron. Jul, 2005 | Pubmed ID: 15996541 Polyglutamine-expanded Ataxin-7 Inhibits STAGA Histone Acetyltransferase Activity to Produce Retinal Degeneration Proceedings of the National Academy of Sciences of the United States of America. Jun, 2005 | Pubmed ID: 15932940 A SCA7 CAG/CTG Repeat Expansion is Stable in Drosophila Melanogaster Despite Modulation of Genomic Context and Gene Dosage Gene. Feb, 2005 | Pubmed ID: 15715978 Dynamic Mutations on the Move in Banff Nature Genetics. Jul, 2004 | Pubmed ID: 15226745 Androgen Receptor YAC Transgenic Mice Recapitulate SBMA Motor Neuronopathy and Implicate VEGF164 in the Motor Neuron Degeneration Neuron. Mar, 2004 | Pubmed ID: 15003169 Interference of Crx-dependent Transcription by Ataxin-7 Involves Interaction Between the Glutamine Regions and Requires the Ataxin-7 Carboxy-terminal Region for Nuclear Localization Human Molecular Genetics. Jan, 2004 | Pubmed ID: 14613968 Polyglutamines Stop Traffic: Axonal Transport As a Common Target in Neurodegenerative Diseases Neuron. Sep, 2003 | Pubmed ID: 14527425 Polyglutamines Placed into Context Neuron. Jun, 2003 | Pubmed ID: 12797953 Familial Dementia with Lewy Bodies with an Atypical Clinical Presentation Journal of Geriatric Psychiatry and Neurology. Mar, 2003 | Pubmed ID: 12641375 Genomic Context Drives SCA7 CAG Repeat Instability, While Expressed SCA7 CDNAs Are Intergenerationally and Somatically Stable in Transgenic Mice Human Molecular Genetics. Jan, 2003 | Pubmed ID: 12490531 Familial Dementia with Lewy Bodies: a Clinical and Neuropathological Study of 2 Families Archives of Neurology. Oct, 2002 | Pubmed ID: 12374501 Polyglutamine and Transcription: Gene Expression Changes Shared by DRPLA and Huntington's Disease Mouse Models Reveal Context-independent Effects Human Molecular Genetics. Aug, 2002 | Pubmed ID: 12165555 Polyglutamine-expanded Ataxin-7 Promotes Non-cell-autonomous Purkinje Cell Degeneration and Displays Proteolytic Cleavage in Ataxic Transgenic Mice The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Jun, 2002 | Pubmed ID: 12077187 Purkinje Cell Degeneration (pcd) Phenotypes Caused by Mutations in the Axotomy-induced Gene, Nna1 Science (New York, N.Y.). Mar, 2002 | Pubmed ID: 11884758 En enkel komposit Fenotyp Scoring System för utvärdering musmodeller av cerebellär ataxi Stephan J. Guyenet1, Stephanie A. Furrer2, Vincent M. Damian1, Travis D. Baughan2, Albert R. La Spada*3, Gwenn A. Garden*2 1Department of Biochemistry, University of Washington, 2Department of Neurology, University of Washington, 3Division of Genetics, Departments of Pediatrics and Cellular and Molecular Medicine, and the Institute for Genomic Medicine, University of California, San Diego - Rady Children’s Hospital JoVE 1787 Neuroscience
En enkel komposit Fenotyp Scoring System för utvärdering musmodeller av cerebellär ataxi Stephan J. Guyenet1, Stephanie A. Furrer2, Vincent M. Damian1, Travis D. Baughan2, Albert R. La Spada*3, Gwenn A. Garden*2 1Department of Biochemistry, University of Washington, 2Department of Neurology, University of Washington, 3Division of Genetics, Departments of Pediatrics and Cellular and Molecular Medicine, and the Institute for Genomic Medicine, University of California, San Diego - Rady Children’s Hospital JoVE 1787 Neuroscience