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Albert La Spada

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Stephanie A Furrer; Gwenn A Garden; Travis D Baughn; Vincent A Damian; Mathini S Mohanachandran; Kien-Thiet Nguyen; Bryce L Sopher; Sarah M Waldherr; Albert R La Spada
Reduction of mutant ataxin-7 expression restores motor function and prevents cerebellar synaptic reorganization in a conditional mouse model of SCA7.
Taiji Tsunemi; Travis D Ashe; Jonathan Au; Vincent A Damian; Eduardo R Lazarowski; Eliezer Masliah; Bradley E Morrison; Ruben A Vázquez Roque; Kathryn R Soriano; Albert R La Spada
PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function.
Gwenn A Garden; Albert R La Spada
Intercellular (mis)communication in neurodegenerative disease.

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All Publications

2013: Stephanie A Furrer; Gwenn A Garden; Travis D Baughn; Vincent A Damian; Mathini S Mohanachandran; Kien-Thiet Nguyen; Bryce L Sopher; Sarah M Waldherr; Albert R La Spada
Reduction of mutant ataxin-7 expression restores motor function and prevents cerebellar synaptic reorganization in a conditional mouse model of SCA7.
Human molecular genetics 2013;22(5):890-903.
2012: Taiji Tsunemi; Travis D Ashe; Jonathan Au; Vincent A Damian; Eduardo R Lazarowski; Eliezer Masliah; Bradley E Morrison; Ruben A Vázquez Roque; Kathryn R Soriano; Albert R La Spada
PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function.
Science translational medicine 2012;4(142):142ra97.
2012: Gwenn A Garden; Albert R La Spada
Intercellular (mis)communication in neurodegenerative disease.
Neuron 2012;73(5):886-901.
2011: Stephanie A Furrer; Gwenn A Garden; Christopher Chang; Vincent A Damian; Mathini S Mohanachandran; Bryce L Sopher; Sarah M Waldherr; Albert R La Spada
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 2011;31(45):16269-78.
2011: Bryce L Sopher; Susan M Sunkin; Cortlandt P Thienes; Paula D Ladd; Randell T Libby; Victor V Pineda; Galina N Filippova; Terry Gaasterland; James B Hurley; Albert R La Spada
CTCF regulates ataxin-7 expression through promotion of a convergently transcribed, antisense noncoding RNA.
Neuron 2011;70(6):1071-84.
2009: Shona Mookerjee; Theodora Papanikolaou; Stephan J Guyenet; Vanitha Sampath; Amy Lin; Cathy Vitelli; Francesco DeGiacomo; Bryce L Sopher; Sylvia F Chen; Albert R La Spada; Lisa M Ellerby
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 2009;29(48):15134-44.
2009: John Ravits; Albert R La Spada
ALS motor phenotype heterogeneity, focality, and spread: deconstructing motor neuron degeneration.
Neurology 2009;73(10):805-11.
2009: Jessica E Young; Albert R La Spada
Development of selective nutrient deprivation as a system to study autophagy induction and regulation in neurons.
Autophagy 2009;5(4):555-7.
2009: Albert R La Spada
Getting a handle on Huntington's disease: silencing neurodegeneration.
Nature medicine 2009;15(3):252-3.
2009: Jessica E Young; Gwenn A Garden; Refugio A Martinez; Fumiaki Tanaka; C Miguel Sandoval; Annette C Smith; Bryce L Sopher; Amy Lin; Kenneth H Fischbeck; Lisa M Ellerby; Richard S Morrison; J. Paul Taylor; Albert R La Spada
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 2009;29(7):1987-97.
2009: Jessica E Young; Refugio A Martinez; Albert R La Spada
Nutrient deprivation induces neuronal autophagy and implicates reduced insulin signaling in neuroprotective autophagy activation.
The Journal of biological chemistry 2009;284(4):2363-73.
2009: Lisa Chakrabarti; Jeremiah Eng; Nishi Ivanov; Gwenn A Garden; Albert R La Spada
Autophagy activation and enhanced mitophagy characterize the Purkinje cells of pcd mice prior to neuronal death.
Molecular brain 2009;2():24.
2008: Randell T Libby; Katharine A Hagerman; Victor V Pineda; Rachel Lau; Diane H Cho; Sandy L Baccam; Michelle M Axford; John D Cleary; James M Moore; Bryce L Sopher; Stephen J Tapscott; Galina N Filippova; Christopher E Pearson; Albert R La Spada
CTCF cis-regulates trinucleotide repeat instability in an epigenetic manner: a novel basis for mutational hot spot determination.
PLoS genetics 2008;4(11):e1000257.
2008: Gregory A Cary; Albert R La Spada
Androgen receptor function in motor neuron survival and degeneration.
Physical medicine and rehabilitation clinics of North America 2008;19(3):479-94, viii.
2008: Gwenn A Garden; Albert R La Spada
Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegeneration.
Cerebellum (London, England) 2008;7(2):138-49.
2007: Christine Marie Clarke; Cara Plata; Bonnie Cole; Karen Tsuchiya; Albert R La Spada; Raj P Kapur
Visceral neuropathy and intestinal pseudo-obstruction in a murine model of a nuclear inclusion disease.
Gastroenterology 2007;133(6):1971-8.
2007: Jessica E Young; Launce Gouw; Stephanie Propp; Bryce L Sopher; Jillian Taylor; Amy Lin; Evan Hermel; Anna Logvinova; Sylvia F Chen; Shiming Chen; Dale E Bredesen; Ray Truant; Louis J Ptacek; Albert R La Spada; Lisa M Ellerby
Proteolytic cleavage of ataxin-7 by caspase-7 modulates cellular toxicity and transcriptional dysregulation.
The Journal of biological chemistry 2007;282(41):30150-60.
2007: Isabella Palazzolo; Barrington Burnett; Jessica E Young; Phebe L Brenne; Albert R La Spada; Kenneth H Fischbeck; Brian Howell; Maria Pennuto
Akt blocks ligand binding and protects against expanded polyglutamine androgen receptor toxicity.
Human molecular genetics 2007;16(13):1593-603.
2006: Albert R La Spada
Neurodegeneration: a case of arrested development?
Cell 2006;127(4):669-71.
2006: Patrick Weydt; Victor V Pineda; Anne E Torrence; Randell T Libby; Terrence F Satterfield; Eduardo R Lazarowski; Merle L Gilbert; Gregory J Morton; Theodor K Bammler; Andrew D Strand; Libin Cui; Richard P Beyer; Courtney N Easley; Annette C Smith; Dimitri Krainc; Serge Luquet; Ian R Sweet; Michael W Schwartz; Albert R La Spada
Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration.
Cell metabolism 2006;4(5):349-62.
2006: Yuxin Fan; Pornprot Limprasert; Ian V J Murray; Annette C Smith; Virginia M-Y Lee; John Q Trojanowski; Bryce L Sopher; Albert R La Spada
Beta-synuclein modulates alpha-synuclein neurotoxicity by reducing alpha-synuclein protein expression.
Human molecular genetics 2006;15(20):3002-11.
2006: Sara K Custer; Gwenn A Garden; Nishi Gill; Udo Rueb; Randell T Libby; Christian Schultz; Stephan J Guyenet; Thomas Deller; Lesnick E Westrum; Bryce L Sopher; Albert R La Spada
Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport.
Nature neuroscience 2006;9(10):1302-11.
2006: Patrick Weydt; Albert R La Spada
Targeting protein aggregation in neurodegeneration--lessons from polyglutamine disorders.
Expert opinion on therapeutic targets 2006;10(4):505-13.
2006: Patrick S Thomas; Gregory S Fraley; Vincent Damian; Vincent Damien; Lillie Woodke; Francisco Zapata; Bryce L Sopher; Stephen R Plymate; Albert R La Spada
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 2006;15(14):2225-38.
2006: Bryce L Sopher; Albert R La Spada
Efficient recombination-based methods for bacterial artificial chromosome fusion and mutagenesis.
Gene 2006;371(1):136-43.
2006: Jillian Taylor; Sara K Grote; Jianrun Xia; Mark Vandelft; Joanna Graczyk; Lisa M Ellerby; Albert R La Spada; Ray Truant
Ataxin-7 can export from the nucleus via a conserved exportin-dependent signal.
The Journal of biological chemistry 2006;281(5):2730-9.
2006: Lisa Chakrabarti; James T Neal; Michael Miles; Refugio A Martinez; Annette C Smith; Bryce L Sopher; Albert R La Spada
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 2006;17(2):103-10.
2005: Albert R La Spada
Huntington's disease and neurogenesis: FGF-2 to the rescue?
Proceedings of the National Academy of Sciences of the United States of America 2005;102(50):17889-90.
2005: Nancy M Bonini; Albert R La Spada
Silencing polyglutamine degeneration with RNAi.
Neuron 2005;48(5):715-8.
2005: Albert R La Spada; Patrick Weydt
Targeting toxic proteins for turnover.
Nature medicine 2005;11(10):1052-3.
2005: Albert R La Spada; Richard S Morrison
The power of the dark side: Huntington's disease protein and p53 form a deadly alliance.
Neuron 2005;47(1):1-3.
2005: Vikas Palhan; Shiming Chen; Guang-Hua Peng; Agneta Tjernberg; Armin Gamper; Yuxin Fan; Brian T Chait; Albert R La Spada; Robert G Roeder
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 2005;102(24):8472-7.
2005: Stephen M Jackson; Alexander J Whitworth; Jessica C Greene; Randell T Libby; Sandy L Baccam; Leo J Pallanck; Albert R La Spada
A SCA7 CAG/CTG repeat expansion is stable in Drosophila melanogaster despite modulation of genomic context and gene dosage.
Gene 2005;347(1):35-41.
2004: H Ohtake; Pornprot Limprasert; Y Fan; Osamu Onodera; Akiyoshi Kakita; Hidenobu Takahashi; Lauren T Bonner; Debby W Tsuang; Ian V J Murray; Virginia M-Y Lee; John Q Trojanowski; A Ishikawa; J Idezuka; M Murata; T Toda; Thomas D Bird; James B Leverenz; Shoji Tsuji; Albert R La Spada
Beta-synuclein gene alterations in dementia with Lewy bodies.
Neurology 2004;63(5):805-11.
2004: Albert R La Spada; Robert I Richards; Bé Wieringa
Dynamic mutations on the move in Banff.
Nature genetics 2004;36(7):667-70.
2004: Bryce L Sopher; Patrick S Thomas; Michelle A LaFevre-Bernt; Ida E Holm; Scott A Wilke; Carol B Ware; Lee-Way Jin; Randell T Libby; Lisa M Ellerby; Albert R La Spada
Androgen receptor YAC transgenic mice recapitulate SBMA motor neuronopathy and implicate VEGF164 in the motor neuron degeneration.
Neuron 2004;41(5):687-99.
2004: Shiming Chen; Guang-Hua Peng; Xuejiao Wang; Annette C Smith; Sara K Grote; Bryce L Sopher; Albert R La Spada
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 2004;13(1):53-67.
2003: Mel B Feany; Albert R La Spada
Polyglutamines stop traffic: axonal transport as a common target in neurodegenerative diseases.
Neuron 2003;40(1):1-2.
2003: Albert R La Spada; J. Paul Taylor
Polyglutamines placed into context.
Neuron 2003;38(5):681-4.
2003: Lauren T Bonner; Debby W Tsuang; Monique M Cherrier; Charisma J Eugenio; Q Du Jennifer; Ellen J Steinbart; Pornprot Limprasert; Albert R La Spada; Benjamin Seltzer; Thomas D Bird; James B Leverenz
Familial dementia with Lewy bodies with an atypical clinical presentation.
Journal of geriatric psychiatry and neurology 2003;16(1):59-64.
2003: Sara K Grote; Albert R La Spada
Insights into the molecular basis of polyglutamine neurodegeneration from studies of a spinocerebellar ataxia type 7 mouse model.
Cytogenetic and genome research 2003;100(1-4):164-74.
2003: Randell T Libby; Darren G Monckton; Ying-Hui Fu; Refugio A Martinez; John P McAbney; R Lau; David D Einum; Kerrie Nichol; Carol B Ware; Louis J Ptacek; Christopher E Pearson; Albert R La Spada
Genomic context drives SCA7 CAG repeat instability, while expressed SCA7 cDNAs are intergenerationally and somatically stable in transgenic mice.
Human molecular genetics 2003;12(1):41-50.
2002: Debby W Tsuang; Aaron M Dalan; Charisma J Eugenio; Parvoneh Poorkaj-Navas; Pornprot Limprasert; Albert R La Spada; Ellen J Steinbart; Thomas D Bird; James B Leverenz
Familial dementia with lewy bodies: a clinical and neuropathological study of 2 families.
Archives of neurology 2002;59(10):1622-30.
2002: Ruth Luthi-Carter; Andrew D Strand; Sarah A Hanson; Charles Kooperberg; Gabriele Schilling; Albert R La Spada; Diane E Merry; Anne B Young; Christopher A Ross; David R Borchelt; James M Olson
Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects.
Human molecular genetics 2002;11(17):1927-37.
2002: Gwenn A Garden; Randell T Libby; Ying-Hui Fu; Yoshito Kinoshita; Jing Huang; Daniel E Possin; Annette C Smith; Refugio A Martinez; Gabriel C Fine; Sara K Grote; Carol B Ware; David D Einum; Richard S Morrison; Louis J Ptacek; Bryce L Sopher; Albert R La Spada
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 2002;22(12):4897-905.
2002: Angeles Fernandez-Gonzalez; Albert R La Spada; Jason Treadaway; Jason C Higdon; Belinda S Harris; Richard L Sidman; James I Morgan; Jian Zuo
Purkinje cell degeneration (pcd) phenotypes caused by mutations in the axotomy-induced gene, Nna1.
Science (New York, N.Y.) 2002;295(5561):1904-6.
2001: Albert R La Spada; Ying-Hui Fu; Bryce L Sopher; Randell T Libby; X Wang; L Y Li; David D Einum; Jing Huang; Daniel E Possin; Annette C Smith; Refugio A Martinez; KL Koszdin; piper meigs treuting; Carol B Ware; James B Hurley; Louis J Ptácek; S Chen
Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7.
Neuron 2001;31(6):913-27.
2001: Bryce L Sopher; KL Koszdin; M E McClain; S B Myrick; Refugio A Martinez; Annette C Smith; Albert R La Spada
Genomic organization, chromosome location, and expression analysis of mouse beta-synuclein, a candidate for involvement in neurodegeneration.
Cytogenetics and cell genetics 2001;93(1-2):117-23.
2000: Bryce L Sopher; S B Myrick; J Y Hong; Annette C Smith; Albert R La Spada
In vivo expansion of trinucleotide repeats yields plasmid and YAC constructs for targeting and transgenesis.
Gene 2000;261(2):383-90.
2000: Magali Fernandez; M E McClain; Refugio A Martinez; K Snow; Hillary Lipe; J Ravits; Thomas D Bird; Albert R La Spada
Late-onset SCA2: 33 CAG repeats are sufficient to cause disease.
Neurology 2000;55(4):569-72.
1998: Albert R La Spada; Kenneth R Peterson; S A Meadows; M E McClain; G Jeng; Renée S Chmelar; H A Haugen; K Chen; MJ Singer; D Moore; Barbara J Trask; Kenneth H Fischbeck; CH Clegg; G S McKnight
Androgen receptor YAC transgenic mice carrying CAG 45 alleles show trinucleotide repeat instability.
Human molecular genetics 1998;7(6):959-67.
1998: WT Hofgärtner; Albert R La Spada; JF Tait
Case of the month: August 1997--a 13 year old girl with progressive movement disorder.
Brain pathology (Zurich, Switzerland) 1998;8(1):237-8.
1997: Albert R La Spada
Trinucleotide repeat instability: genetic features and molecular mechanisms.
Brain pathology (Zurich, Switzerland) 1997;7(3):943-63.
1997: Albert R La Spada; A W Clark
Inherited neurodegenerative disorders caused by CAG/polyglutamine tract expansions: symposium introduction.
Brain pathology (Zurich, Switzerland) 1997;7(3):877-80.
1996: R Spiegel; Albert R La Spada; W Kress; Kenneth H Fischbeck; W Schmid
Somatic stability of the expanded CAG trinucleotide repeat in X-linked spinal and bulbar muscular atrophy.
Human mutation 1996;8(1):32-7.
1995: Albert R La Spada; B S Skålhegg; R Henderson; G Schmer; R Pierce; W Chandler
Brief report: fatal hemorrhage in a patient with an acquired inhibitor of human thrombin.
The New England journal of medicine 1995;333(8):494-7.
1994: Albert R La Spada; Henry L Paulson; Kenneth H Fischbeck
Trinucleotide repeat expansion in neurological disease.
Annals of neurology 1994;36(6):814-22.
1994: Adrian Danek; T N Witt; Klaus Mann; Hans-Udo Schweikert; Gabriela Romalo; Albert R La Spada; Kenneth H Fischbeck
Decrease in androgen binding and effect of androgen treatment in a case of X-linked bulbospinal neuronopathy.
The Clinical investigator 1994;72(11):892-7.
1993: D B Roling; Albert R La Spada; Kenneth H Fischbeck
Kennedy's disease.
Neurology 1993;43(11):2424-5.
1992: Albert R La Spada; D B Roling; A E Harding; C L Warner; R Spiegel; I Hausmanowa-Petrusewicz; W C Yee; Kenneth H Fischbeck
Meiotic stability and genotype-phenotype correlation of the trinucleotide repeat in X-linked spinal and bulbar muscular atrophy.
Nature genetics 1992;2(4):301-4.
1991: Albert R La Spada; Elizabeth M Wilson; D B Lubahn; A E Harding; Kenneth H Fischbeck
Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy.
Nature 1991;352(6330):77-9.
1991: Kenneth H Fischbeck; D Souders; Albert R La Spada
A candidate gene for X-linked spinal muscular atrophy.
Advances in neurology 1991;56():209-13.