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Al La Spada, MD PhD - Publications

1993-2009 University of Washington, Seattle, Seattle, WA 
 2009- Pediatrics University of California, San Diego, La Jolla, CA 
Polyglutamine disease mechanisms

111 high-probability publications. We are testing a new system for linking publications to authors. You can help! If you notice any inaccuracies, please sign in and mark papers as correct or incorrect matches. If you identify any major omissions or other inaccuracies in the publication list, please let us know.

Year Citation  Score
2018 Badders NM, Korff A, Miranda HC, Vuppala PK, Smith RB, Winborn BJ, Quemin ER, Sopher BL, Dearman J, Messing J, Kim NC, Moore J, Freibaum BD, Kanagaraj AP, Fan B, ... ... La Spada AR, et al. Selective modulation of the androgen receptor AF2 domain rescues degeneration in spinal bulbar muscular atrophy. Nature Medicine. PMID 29505030 DOI: 10.1038/Nm.4500  0.84
2016 Polanco MJ, Parodi S, Piol D, Stack C, Chivet M, Contestabile A, Miranda HC, Lievens PM, Espinoza S, Jochum T, Rocchi A, Grunseich C, Gainetdinov RR, Cato AC, Lieberman AP, ... La Spada AR, et al. Adenylyl cyclase activating polypeptide reduces phosphorylation and toxicity of the polyglutamine-expanded androgen receptor in spinobulbar muscular atrophy. Science Translational Medicine. 8: 370ra181. PMID 28003546 DOI: 10.1126/scitranslmed.aaf9526  0.84
2015 Guyenet SJ, Mookerjee SS, Lin A, Custer SK, Chen SF, Sopher BL, La Spada AR, Ellerby LM. Proteolytic cleavage of ataxin-7 promotes SCA7 retinal degeneration and neurological dysfunction. Human Molecular Genetics. 24: 3908-17. PMID 25859008 DOI: 10.1093/Hmg/Ddv121  0.84
2015 Cortes CJ, La Spada AR. Autophagy in polyglutamine disease: Imposing order on disorder or contributing to the chaos? Molecular and Cellular Neurosciences. 66: 53-61. PMID 25771431 DOI: 10.1016/j.mcn.2015.03.010  0.84
2015 Ward JM, La Spada AR. Ataxin-3, DNA damage repair, and SCA3 cerebellar degeneration: on the path to parsimony? Plos Genetics. 11: e1004937. PMID 25633989 DOI: 10.1371/journal.pgen.1004937  0.84
2015 Eschbach J, von Einem B, Müller K, Bayer H, Scheffold A, Morrison BE, Rudolph KL, Thal DR, Witting A, Weydt P, Otto M, Fauler M, Liss B, McLean PJ, Spada AR, et al. Mutual exacerbation of peroxisome proliferator-activated receptor γ coactivator 1α deregulation and α-synuclein oligomerization. Annals of Neurology. 77: 15-32. PMID 25363075 DOI: 10.1002/Ana.24294  0.84
2014 Dubinsky AN, Dastidar SG, Hsu CL, Zahra R, Djakovic SN, Duarte S, Esau CC, Spencer B, Ashe TD, Fischer KM, MacKenna DA, Sopher BL, Masliah E, Gaasterland T, Chau BN, ... ... La Spada AR, et al. Let-7 coordinately suppresses components of the amino acid sensing pathway to repress mTORC1 and induce autophagy. Cell Metabolism. 20: 626-38. PMID 25295787 DOI: 10.1016/J.Cmet.2014.09.001  0.84
2014 Ward JM, La Spada AR. Identification of the SCA21 disease gene: remaining challenges and promising opportunities. Brain : a Journal of Neurology. 137: 2626-8. PMID 25217789 DOI: 10.1093/brain/awu217  0.84
2014 Cortes CJ, Miranda HC, Frankowski H, Batlevi Y, Young JE, Le A, Ivanov N, Sopher BL, Carromeu C, Muotri AR, Garden GA, La Spada AR. Polyglutamine-expanded androgen receptor interferes with TFEB to elicit autophagy defects in SBMA. Nature Neuroscience. 17: 1180-9. PMID 25108912 DOI: 10.1038/Nn.3787  0.52
2014 La Spada AR. Something wicked this way comes: huntingtin. Nature Neuroscience. 17: 1014-5. PMID 25065434 DOI: 10.1038/nn.3770  0.84
2014 Ramachandran PS, Boudreau RL, Schaefer KA, La Spada AR, Davidson BL. Nonallele specific silencing of ataxin-7 improves disease phenotypes in a mouse model of SCA7. Molecular Therapy : the Journal of the American Society of Gene Therapy. 22: 1635-42. PMID 24930601 DOI: 10.1038/Mt.2014.108  0.84
2014 Montague K, Malik B, Gray AL, La Spada AR, Hanna MG, Szabadkai G, Greensmith L. Endoplasmic reticulum stress in spinal and bulbar muscular atrophy: a potential target for therapy. Brain : a Journal of Neurology. 137: 1894-906. PMID 24898351 DOI: 10.1093/Brain/Awu114  0.84
2014 Lieberman AP, Yu Z, Murray S, Peralta R, Low A, Guo S, Yu XX, Cortes CJ, Bennett CF, Monia BP, La Spada AR, Hung G. Peripheral androgen receptor gene suppression rescues disease in mouse models of spinal and bulbar muscular atrophy. Cell Reports. 7: 774-84. PMID 24746732 DOI: 10.1016/j.celrep.2014.02.008  0.84
2014 Cortes CJ, Ling SC, Guo LT, Hung G, Tsunemi T, Ly L, Tokunaga S, Lopez E, Sopher BL, Bennett CF, Shelton GD, Cleveland DW, La Spada AR. Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy. Neuron. 82: 295-307. PMID 24742458 DOI: 10.1016/J.Neuron.2014.03.001  0.84
2014 Cortes CJ, La Spada AR. The many faces of autophagy dysfunction in Huntington's disease: from mechanism to therapy. Drug Discovery Today. 19: 963-71. PMID 24632005 DOI: 10.1016/j.drudis.2014.02.014  0.84
2014 Mason AG, Tomé S, Simard JP, Libby RT, Bammler TK, Beyer RP, Morton AJ, Pearson CE, La Spada AR. Expression levels of DNA replication and repair genes predict regional somatic repeat instability in the brain but are not altered by polyglutamine disease protein expression or age. Human Molecular Genetics. 23: 1606-18. PMID 24191263 DOI: 10.1093/Hmg/Ddt551  0.84
2013 Bennett CL, Chen Y, Vignali M, Lo RS, Mason AG, Unal A, Huq Saifee NP, Fields S, La Spada AR. Protein interaction analysis of senataxin and the ALS4 L389S mutant yields insights into senataxin post-translational modification and uncovers mutant-specific binding with a brain cytoplasmic RNA-encoded peptide. Plos One. 8: e78837. PMID 24244371 DOI: 10.1371/Journal.Pone.0078837  0.84
2013 Sekigawa A, Sekiyama K, Fujita M, Takamatsu Y, La Spada AR, Masliah E, Hashimoto M. Dual effects of β-synuclein on the pathogenesis of Parkinson disease. Annals of Neurology. 74: 306. PMID 23836414 DOI: 10.1002/Ana.23936  0.84
2013 Fujita K, Nakamura Y, Oka T, Ito H, Tamura T, Tagawa K, Sasabe T, Katsuta A, Motoki K, Shiwaku H, Sone M, Yoshida C, Katsuno M, Eishi Y, Murata M, ... ... La Spada AR, et al. A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases. Nature Communications. 4: 1816. PMID 23652004 DOI: 10.1038/Ncomms2828  0.84
2013 Haun F, Nakamura T, Shiu AD, Cho DH, Tsunemi T, Holland EA, La Spada AR, Lipton SA. S-nitrosylation of dynamin-related protein 1 mediates mutant huntingtin-induced mitochondrial fragmentation and neuronal injury in Huntington's disease. Antioxidants & Redox Signaling. 19: 1173-84. PMID 23641925 DOI: 10.1089/ars.2012.4928  0.84
2013 Malik B, Nirmalananthan N, Gray AL, La Spada AR, Hanna MG, Greensmith L. Co-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: implications for therapy. Brain : a Journal of Neurology. 136: 926-43. PMID 23393146 DOI: 10.1093/Brain/Aws343  0.84
2013 Furrer SA, Waldherr SM, Mohanachandran MS, Baughn TD, Nguyen KT, Sopher BL, Damian VA, Garden GA, La Spada AR. Reduction of mutant ataxin-7 expression restores motor function and prevents cerebellar synaptic reorganization in a conditional mouse model of SCA7. Human Molecular Genetics. 22: 890-903. PMID 23197655 DOI: 10.1093/Hmg/Dds495  0.84
2013 Fratta P, Malik B, Gray A, La Spada AR, Hanna MG, Fisher EM, Greensmith L. FUS is not dysregulated by the spinal bulbar muscular atrophy androgen receptor polyglutamine repeat expansion. Neurobiology of Aging. 34: 1516.e17-9. PMID 23062703 DOI: 10.1016/J.Neurobiolaging.2012.09.008  0.84
2012 Sekigawa A, Fujita M, Sekiyama K, Takamatsu Y, Hatano T, Rockenstein E, La Spada AR, Masliah E, Hashimoto M. Distinct mechanisms of axonal globule formation in mice expressing human wild type α-synuclein or dementia with Lewy bodies-linked P123H β-synuclein. Molecular Brain. 5: 34. PMID 23013868 DOI: 10.1186/1756-6606-5-34  0.84
2012 Ward JM, La Spada AR. The expanding world of stem cell modeling of Huntington's disease: creating tools with a promising future. Genome Medicine. 4: 68. PMID 22943447 DOI: 10.1186/gm369  0.84
2012 La Spada AR. PPARGC1A/PGC-1α, TFEB and enhanced proteostasis in Huntington disease: defining regulatory linkages between energy production and protein-organelle quality control. Autophagy. 8: 1845-7. PMID 22932698 DOI: 10.4161/auto.21862  0.84
2012 Tsunemi T, Ashe TD, Morrison BE, Soriano KR, Au J, Roque RA, Lazarowski ER, Damian VA, Masliah E, La Spada AR. PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function. Science Translational Medicine. 4: 142ra97. PMID 22786682 DOI: 10.1126/Scitranslmed.3003799  0.84
2012 Garden GA, La Spada AR. Intercellular (mis)communication in neurodegenerative disease. Neuron. 73: 886-901. PMID 22405200 DOI: 10.1016/J.Neuron.2012.02.017  0.84
2012 La Spada AR. Finding a sirtuin truth in Huntington's disease. Nature Medicine. 18: 24-6. PMID 22227661 DOI: 10.1038/nm.2624  0.84
2012 Nakamura Y, Tagawa K, Oka T, Sasabe T, Ito H, Shiwaku H, La Spada AR, Okazawa H. Ataxin-7 associates with microtubules and stabilizes the cytoskeletal network. Human Molecular Genetics. 21: 1099-110. PMID 22100762 DOI: 10.1093/hmg/ddr539  0.84
2012 Tsunemi T, La Spada AR. PGC-1α at the intersection of bioenergetics regulation and neuron function: from Huntington's disease to Parkinson's disease and beyond. Progress in Neurobiology. 97: 142-51. PMID 22100502 DOI: 10.1016/j.pneurobio.2011.10.004  0.84
2012 Hashimoto M, La Spada AR. β-synuclein in the pathogenesis of Parkinson's disease and related α-synucleinopathies: Emerging roles and new directions Future Neurology. 7: 155-163. DOI: 10.2217/fnl.12.5  0.84
2012 Zahra R, La Spada AR. Cellular and Molecular Basis of Neurodegeneration in the CAG-Polyglutamine Repeat Diseases Basic Neurochemistry. 844-855. DOI: 10.1016/B978-0-12-374947-5.00048-1  0.84
2011 Furrer SA, Mohanachandran MS, Waldherr SM, Chang C, Damian VA, Sopher BL, Garden GA, La Spada AR. 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. 31: 16269-78. PMID 22072678 DOI: 10.1523/Jneurosci.4000-11.2011  0.84
2011 Wilburn B, Rudnicki DD, Zhao J, Weitz TM, Cheng Y, Gu X, Greiner E, Park CS, Wang N, Sopher BL, La Spada AR, Osmand A, Margolis RL, Sun YE, Yang XW. An antisense CAG repeat transcript at JPH3 locus mediates expanded polyglutamine protein toxicity in Huntington's disease-like 2 mice. Neuron. 70: 427-40. PMID 21555070 DOI: 10.1016/J.Neuron.2011.03.021  0.84
2011 Malik B, Nirmalananthan N, Bilsland LG, La Spada AR, Hanna MG, Schiavo G, Gallo JM, Greensmith L. Absence of disturbed axonal transport in spinal and bulbar muscular atrophy. Human Molecular Genetics. 20: 1776-86. PMID 21317158 DOI: 10.1093/Hmg/Ddr061  0.84
2011 Batlevi Y, La Spada AR. Mitochondrial autophagy in neural function, neurodegenerative disease, neuron cell death, and aging. Neurobiology of Disease. 43: 46-51. PMID 20887789 DOI: 10.1016/j.nbd.2010.09.009  0.84
2010 Kazemi-Esfarjani P, La Spada AR. Déjà vu with a twist: transglutaminases in bioenergetics and transcriptional dysfunction in Huntington's disease. Embo Molecular Medicine. 2: 335-7. PMID 20730854 DOI: 10.1002/emmm.201000092  0.84
2010 Wegrzyn JL, Bark SJ, Funkelstein L, Mosier C, Yap A, Kazemi-Esfarjani P, La Spada AR, Sigurdson C, O'Connor DT, Hook V. Proteomics of dense core secretory vesicles reveal distinct protein categories for secretion of neuroeffectors for cell-cell communication. Journal of Proteome Research. 9: 5002-24. PMID 20695487 DOI: 10.1021/Pr1003104  0.84
2010 Chakrabarti L, Zahra R, Jackson SM, Kazemi-Esfarjani P, Sopher BL, Mason AG, Toneff T, Ryu S, Shaffer S, Kansy JW, Eng J, Merrihew G, MacCoss MJ, Murphy A, Goodlett DR, ... ... La Spada AR, et al. Mitochondrial dysfunction in NnaD mutant flies and Purkinje cell degeneration mice reveals a role for Nna proteins in neuronal bioenergetics. Neuron. 66: 835-47. PMID 20620870 DOI: 10.1016/J.Neuron.2010.05.024  0.84
2010 Guyenet SJ, Furrer SA, Damian VM, Baughan TD, La Spada AR, Garden GA. A simple composite phenotype scoring system for evaluating mouse models of cerebellar ataxia. Journal of Visualized Experiments : Jove. PMID 20495529 DOI: 10.3791/1787  0.84
2010 La Spada A, Ranum LP. Molecular genetic advances in neurological disease: special review issue. Human Molecular Genetics. 19: R1-3. PMID 20484169 DOI: 10.1093/hmg/ddq193  0.84
2010 La Spada AR, Taylor JP. Repeat expansion disease: progress and puzzles in disease pathogenesis. Nature Reviews. Genetics. 11: 247-58. PMID 20177426 DOI: 10.1038/Nrg2748  0.84
2010 Rabin SJ, Kim JM, Baughn M, Libby RT, Kim YJ, Fan Y, Libby RT, La Spada A, Stone B, Ravits J. Sporadic ALS has compartment-specific aberrant exon splicing and altered cell-matrix adhesion biology. Human Molecular Genetics. 19: 313-28. PMID 19864493 DOI: 10.1093/Hmg/Ddp498  0.84
2009 La Spada AR. Memantine strikes the perfect balance. Nature Medicine. 15: 1355-6. PMID 19966768 DOI: 10.1038/nm1209-1355  0.84
2009 Mookerjee S, Papanikolaou T, Guyenet SJ, Sampath V, Lin A, Vitelli C, DeGiacomo F, Sopher BL, Chen SF, La Spada AR, Ellerby LM. 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. 29: 15134-44. PMID 19955365 DOI: 10.1523/Jneurosci.4720-09.2009  0.84
2009 Ravits JM, La Spada AR. ALS motor phenotype heterogeneity, focality, and spread: deconstructing motor neuron degeneration. Neurology. 73: 805-11. PMID 19738176 DOI: 10.1212/WNL.0b013e3181b6bbbd  0.84
2009 Young JE, La Spada AR. Development of selective nutrient deprivation as a system to study autophagy induction and regulation in neurons. Autophagy. 5: 555-7. PMID 19363305 DOI: 10.4161/auto.5.4.8389  0.84
2009 La Spada AR. Getting a handle on Huntington's disease: silencing neurodegeneration. Nature Medicine. 15: 252-3. PMID 19265826 DOI: 10.1038/nm0309-252  0.84
2009 Young JE, Garden GA, Martinez RA, Tanaka F, Sandoval CM, Smith AC, Sopher BL, Lin A, Fischbeck KH, Ellerby LM, Morrison RS, Taylor JP, La Spada AR. 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. 29: 1987-97. PMID 19228953 DOI: 10.1523/Jneurosci.4072-08.2009  0.84
2009 Young JE, Martinez RA, La Spada AR. Nutrient deprivation induces neuronal autophagy and implicates reduced insulin signaling in neuroprotective autophagy activation. The Journal of Biological Chemistry. 284: 2363-73. PMID 19017649 DOI: 10.1074/jbc.M806088200  0.84
2009 La Spada AR. Inducing PGC-lα expression in huntington's disease may slow neuronal dysfunction and neurodegeneration P and T. 34: 94.  0.84
2008 Libby RT, Hagerman KA, Pineda VV, Lau R, Cho DH, Baccam SL, Axford MM, Cleary JD, Moore JM, Sopher BL, Tapscott SJ, Filippova GN, Pearson CE, La Spada AR. CTCF cis-regulates trinucleotide repeat instability in an epigenetic manner: a novel basis for mutational hot spot determination. Plos Genetics. 4: e1000257. PMID 19008940 DOI: 10.1371/Journal.Pgen.1000257  0.84
2008 Cary GA, La Spada AR. Androgen receptor function in motor neuron survival and degeneration. Physical Medicine and Rehabilitation Clinics of North America. 19: 479-94, viii. PMID 18625411 DOI: 10.1016/J.Pmr.2008.03.002  0.84
2008 Garden GA, La Spada AR. Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegeneration. Cerebellum (London, England). 7: 138-49. PMID 18418675 DOI: 10.1007/S12311-008-0027-Y  0.84
2008 Rüb U, Brunt ER, Seidel K, Gierga K, Mooy CM, Kettner M, Van Broeckhoven C, Bechmann I, La Spada AR, Schöls L, den Dunnen W, de Vos RA, Deller T. Spinocerebellar ataxia type 7 (SCA7): widespread brain damage in an adult-onset patient with progressive visual impairments in comparison with an adult-onset patient without visual impairments. Neuropathology and Applied Neurobiology. 34: 155-68. PMID 17971076 DOI: 10.1111/j.1365-2990.2007.00882.x  0.84
2008 Young JE, Gouw L, Propp S, Sopher BL, Taylor J, Lin A, Hermel E, Logvinova A, Chen SF, Chen S, Bredesen DE, Truant R, Ptacek LJ, La Spada AR, Ellerby LM. Proteolytic cleavage of ataxin-7 by caspase-7 modulates cellular toxicity and transcriptional dysregulation (Journal of Biological Chemistry (2007) 282, (30150-30160)) Journal of Biological Chemistry. 283: 16960.  0.84
2007 Clarke CM, Plata C, Cole B, Tsuchiya K, La Spada AR, Kapur RP. Visceral neuropathy and intestinal pseudo-obstruction in a murine model of a nuclear inclusion disease. Gastroenterology. 133: 1971-8. PMID 18054568 DOI: 10.1053/j.gastro.2007.08.043  0.84
2007 Young JE, Gouw L, Propp S, Sopher BL, Taylor J, Lin A, Hermel E, Logvinova A, Chen SF, Chen S, Bredesen DE, Truant R, Ptacek LJ, La Spada AR, Ellerby LM. Proteolytic cleavage of ataxin-7 by caspase-7 modulates cellular toxicity and transcriptional dysregulation. The Journal of Biological Chemistry. 282: 30150-60. PMID 17646170 DOI: 10.1074/Jbc.M705265200  0.84
2007 Palazzolo I, Burnett BG, Young JE, Brenne PL, La Spada AR, Fischbeck KH, Howell BW, Pennuto M. Akt blocks ligand binding and protects against expanded polyglutamine androgen receptor toxicity. Human Molecular Genetics. 16: 1593-603. PMID 17470458 DOI: 10.1093/Hmg/Ddm109  0.84
2007 La Spada AR. Bidirectional expression of CUG and CAG expansion transcripts and intranuclear polyglutamine inclusions in spinocerebellar ataxia type 8 Chemtracts. 19: 118-119.  0.84
2007 La Spada AR. Onset and progression in inherited ALS determined by motor neurons and microglia Chemtracts. 19: 120-121.  0.84
2006 La Spada AR. Neurodegeneration: a case of arrested development? Cell. 127: 669-71. PMID 17110325 DOI: 10.1016/j.cell.2006.11.010  0.84
2006 Weydt P, Pineda VV, Torrence AE, Libby RT, Satterfield TF, Lazarowski ER, Gilbert ML, Morton GJ, Bammler TK, Strand AD, Cui L, Beyer RP, Easley CN, Smith AC, Krainc D, ... ... La Spada AR, et al. Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration. Cell Metabolism. 4: 349-62. PMID 17055784 DOI: 10.1016/J.Cmet.2006.10.004  0.84
2006 Fan Y, Limprasert P, Murray IV, Smith AC, Lee VM, Trojanowski JQ, Sopher BL, La Spada AR. Beta-synuclein modulates alpha-synuclein neurotoxicity by reducing alpha-synuclein protein expression. Human Molecular Genetics. 15: 3002-11. PMID 16959793 DOI: 10.1093/Hmg/Ddl242  0.84
2006 Custer SK, Garden GA, Gill N, Rueb U, Libby RT, Schultz C, Guyenet SJ, Deller T, Westrum LE, Sopher BL, La Spada AR. Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport. Nature Neuroscience. 9: 1302-11. PMID 16936724 DOI: 10.1038/Nn1750  0.84
2006 Weydt P, La Spada AR. Targeting protein aggregation in neurodegeneration--lessons from polyglutamine disorders. Expert Opinion On Therapeutic Targets. 10: 505-13. PMID 16848688 DOI: 10.1517/14728222.10.4.505  0.84
2006 Thomas PS, Fraley GS, Damian V, Damien V, Woodke LB, Zapata F, Sopher BL, Plymate SR, La Spada AR. 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. 15: 2225-38. PMID 16772330 DOI: 10.1093/hmg/ddl148  0.84
2006 Sopher BL, La Spada AR. Efficient recombination-based methods for bacterial artificial chromosome fusion and mutagenesis. Gene. 371: 136-43. PMID 16487669 DOI: 10.1016/j.gene.2005.11.034  0.84
2006 Chakrabarti L, Neal JT, Miles M, Martinez RA, Smith AC, Sopher BL, La Spada AR. 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. 17: 103-10. PMID 16465590 DOI: 10.1007/S00335-005-0096-X  0.84
2006 Taylor J, Grote SK, Xia J, Vandelft M, Graczyk J, Ellerby LM, La Spada AR, Truant R. Ataxin-7 can export from the nucleus via a conserved exportin-dependent signal. The Journal of Biological Chemistry. 281: 2730-9. PMID 16314424 DOI: 10.1074/Jbc.M506751200  0.84
2006 Garden GA, Truant R, Ellerby LM, La Spada AR. Spinocerebellar ataxia type 7: Clinical features to cellular pathogenesis Genetic Instabilities and Neurological Diseases, Second Edition. 399-416. DOI: 10.1016/B978-012369462-1/50028-4  0.84
2005 Bonini NM, La Spada AR. Silencing polyglutamine degeneration with RNAi. Neuron. 48: 715-8. PMID 16337909 DOI: 10.1016/j.neuron.2005.11.008  0.84
2005 La Spada AR. Huntington's disease and neurogenesis: FGF-2 to the rescue? Proceedings of the National Academy of Sciences of the United States of America. 102: 17889-90. PMID 16330780 DOI: 10.1073/pnas.0509222102  0.84
2005 La Spada AR, Weydt P. Targeting toxic proteins for turnover. Nature Medicine. 11: 1052-3. PMID 16211038 DOI: 10.1038/nm1005-1052  0.84
2005 La Spada AR, Morrison RS. The power of the dark side: Huntington's disease protein and p53 form a deadly alliance. Neuron. 47: 1-3. PMID 15996541 DOI: 10.1016/J.Neuron.2005.06.023  0.84
2005 Palhan VB, Chen S, Peng GH, Tjernberg A, Gamper AM, Fan Y, Chait BT, La Spada AR, Roeder RG. 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. 102: 8472-7. PMID 15932940 DOI: 10.1073/Pnas.0503505102  0.84
2005 Jackson SM, Whitworth AJ, Greene JC, Libby RT, Baccam SL, Pallanck LJ, La Spada AR. A SCA7 CAG/CTG repeat expansion is stable in Drosophila melanogaster despite modulation of genomic context and gene dosage. Gene. 347: 35-41. PMID 15715978 DOI: 10.1016/j.gene.2004.12.008  0.84
2005 Thomas PS, La Spada AR. Spinal and bulbar muscular atrophy (Kennedy's disease): A sex-limited, polyglutamine repeat expansion disorder Neurodegenerative Diseases: Neurobiology, Pathogenesis and Therapeutics. 803-816. DOI: 10.1017/CBO9780511544873.055  0.84
2005 La Spada AR. Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization; inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death; pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders Chemtracts. 18: 159-167.  0.84
2004 Ohtake H, Limprasert P, Fan Y, Onodera O, Kakita A, Takahashi H, Bonner LT, Tsuang DW, Murray IV, Lee VM, Trojanowski JQ, Ishikawa A, Idezuka J, Murata M, Toda T, ... ... La Spada AR, et al. Beta-synuclein gene alterations in dementia with Lewy bodies. Neurology. 63: 805-11. PMID 15365127 DOI: 10.1212/01.Wnl.0000139870.14385.3C  0.84
2004 La Spada AR, Richards RI, Wieringa B. Dynamic mutations on the move in Banff. Nature Genetics. 36: 667-70. PMID 15226745 DOI: 10.1038/ng0704-667  0.84
2004 Sopher BL, Thomas PS, LaFevre-Bernt MA, Holm IE, Wilke SA, Ware CB, Jin LW, Libby RT, Ellerby LM, La Spada AR. Androgen receptor YAC transgenic mice recapitulate SBMA motor neuronopathy and implicate VEGF164 in the motor neuron degeneration. Neuron. 41: 687-99. PMID 15003169 DOI: 10.1016/S0896-6273(04)00082-0  0.84
2004 Chen S, Peng GH, Wang X, Smith AC, Grote SK, Sopher BL, La Spada AR. 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. 13: 53-67. PMID 14613968 DOI: 10.1093/Hmg/Ddh005  0.84
2003 Feany MB, La Spada AR. Polyglutamines stop traffic: axonal transport as a common target in neurodegenerative diseases. Neuron. 40: 1-2. PMID 14527425 DOI: 10.1016/S0896-6273(03)00600-7  0.84
2003 Grote SK, La Spada AR. Insights into the molecular basis of polyglutamine neurodegeneration from studies of a spinocerebellar ataxia type 7 mouse model. Cytogenetic and Genome Research. 100: 164-74. PMID 14526177 DOI: 10.1159/000072851  0.84
2003 La Spada AR, Taylor JP. Polyglutamines placed into context. Neuron. 38: 681-4. PMID 12797953 DOI: 10.1016/S0896-6273(03)00328-3  0.84
2003 Bonner LT, Tsuang DW, Cherrier MM, Eugenio CJ, Du Jennifer Q, Steinbart EJ, Limprasert P, La Spada AR, Seltzer B, Bird TD, Leverenz JB. Familial dementia with Lewy bodies with an atypical clinical presentation. Journal of Geriatric Psychiatry and Neurology. 16: 59-64. PMID 12641375 DOI: 10.1177/0891988702250585  0.84
2003 Libby RT, Monckton DG, Fu YH, Martinez RA, McAbney JP, Lau R, Einum DD, Nichol K, Ware CB, Ptacek LJ, Pearson CE, La Spada AR. Genomic context drives SCA7 CAG repeat instability, while expressed SCA7 cDNAs are intergenerationally and somatically stable in transgenic mice. Human Molecular Genetics. 12: 41-50. PMID 12490531 DOI: 10.1093/Hmg/Ddg006  0.84
2002 Tsuang DW, Dalan AM, Eugenio CJ, Poorkaj P, Limprasert P, La Spada AR, Steinbart EJ, Bird TD, Leverenz JB. Familial dementia with lewy bodies: a clinical and neuropathological study of 2 families. Archives of Neurology. 59: 1622-30. PMID 12374501 DOI: 10.1001/Archneur.59.10.1622  0.84
2002 Luthi-Carter R, Strand AD, Hanson SA, Kooperberg C, Schilling G, La Spada AR, Merry DE, Young AB, Ross CA, Borchelt DR, Olson JM. Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects. Human Molecular Genetics. 11: 1927-37. PMID 12165555 DOI: 10.1093/Hmg/11.17.1927  0.84
2002 Garden GA, Libby RT, Fu YH, Kinoshita Y, Huang J, Possin DE, Smith AC, Martinez RA, Fine GC, Grote SK, Ware CB, Einum DD, Morrison RS, Ptacek LJ, Sopher BL, ... La Spada AR, et al. 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. 22: 4897-905. PMID 12077187 DOI: 10.1523/Jneurosci.22-12-04897.2002  0.84
2002 Fernandez-Gonzalez A, La Spada AR, Treadaway J, Higdon JC, Harris BS, Sidman RL, Morgan JI, Zuo J. Purkinje cell degeneration (pcd) phenotypes caused by mutations in the axotomy-induced gene, Nna1. Science (New York, N.Y.). 295: 1904-6. PMID 11884758 DOI: 10.1126/Science.1068912  0.84
2001 La Spada AR, Fu YH, Sopher BL, Libby RT, Wang X, Li LY, Einum DD, Huang J, Possin DE, Smith AC, Martinez RA, Koszdin KL, Treuting PM, Ware CB, Hurley JB, et al. Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7. Neuron. 31: 913-27. PMID 11580893 DOI: 10.1016/S0896-6273(01)00422-6  0.84
2001 Matsumura R, Futamura N, La Spada AR, Bird TD. Late-onset SCA2: 33 CAG repeats are sufficient to cause disease [3] (multiple letters) Neurology. 57: 566. PMID 11502947 DOI: 10.1212/Wnl.57.3.566  0.84
2001 Sopher BL, Koszdin KL, McClain ME, Myrick SB, Martinez RA, Smith AC, La Spada AR. Genomic organization, chromosome location, and expression analysis of mouse beta-synuclein, a candidate for involvement in neurodegeneration. Cytogenetics and Cell Genetics. 93: 117-23. PMID 11474193  0.84
2000 Sopher BL, Myrick SB, Hong JY, Smith AC, La Spada AR. In vivo expansion of trinucleotide repeats yields plasmid and YAC constructs for targeting and transgenesis. Gene. 261: 383-90. PMID 11167027 DOI: 10.1016/S0378-1119(00)00508-4  0.84
2000 Fernandez M, McClain ME, Martinez RA, Snow K, Lipe H, Ravits J, Bird TD, La Spada AR. Late-onset SCA2: 33 CAG repeats are sufficient to cause disease. Neurology. 55: 569-72. PMID 10953195  0.84
1999 La Spada AR. Repeat meeting's repeat performance Trends in Genetics. 15: 350-351. PMID 10532895 DOI: 10.1016/S0168-9525(99)01806-5  0.84
1998 La Spada AR, Peterson KR, Meadows SA, McClain ME, Jeng G, Chmelar RS, Haugen HA, Chen K, Singer MJ, Moore D, Trask BJ, Fischbeck KH, Clegg CH, McKnight GS. Androgen receptor YAC transgenic mice carrying CAG 45 alleles show trinucleotide repeat instability. Human Molecular Genetics. 7: 959-67. PMID 9580659 DOI: 10.1093/hmg/7.6.959  0.84
1998 Hofgärtner WT, La Spada AR, Tait JF. Case of the month: August 1997--a 13 year old girl with progressive movement disorder. Brain Pathology (Zurich, Switzerland). 8: 237-8. PMID 9458182  0.84
1997 La Spada AR. Trinucleotide repeat instability: genetic features and molecular mechanisms. Brain Pathology (Zurich, Switzerland). 7: 943-63. PMID 9217977  0.84
1997 La Spada AR, Clark AW. Inherited neurodegenerative disorders caused by CAG/polyglutamine tract expansions: symposium introduction. Brain Pathology (Zurich, Switzerland). 7: 877-80. PMID 9217973  0.84
1996 Spiegel R, La Spada AR, Kress W, Fischbeck KH, Schmid W. Somatic stability of the expanded CAG trinucleotide repeat in X-linked spinal and bulbar muscular atrophy. Human Mutation. 8: 32-7. PMID 8807333 DOI: 10.1002/(SICI)1098-1004(1996)8:1<32::AID-HUMU4>3.0.CO;2-R  0.84
1995 La Spada AR, SkÃ¥lhegg BS, Henderson R, Schmer G, Pierce R, Chandler W. Brief report: fatal hemorrhage in a patient with an acquired inhibitor of human thrombin. The New England Journal of Medicine. 333: 494-7. PMID 7623881 DOI: 10.1056/NEJM199508243330805  0.84
1994 La Spada AR, Paulson HL, Fischbeck KH. Trinucleotide repeat expansion in neurological disease. Annals of Neurology. 36: 814-22. PMID 7998766 DOI: 10.1002/ana.410360604  0.84
1994 Danek A, Witt TN, Mann K, Schweikert HU, Romalo G, La Spada AR, Fischbeck KH. Decrease in androgen binding and effect of androgen treatment in a case of X-linked bulbospinal neuronopathy. The Clinical Investigator. 72: 892-7. PMID 7894219 DOI: 10.1007/BF00190748  0.84
1993 Roling DB, La Spada AR, Fischbeck KH. Kennedy's disease. Neurology. 43: 2424-5. PMID 8232979  1
1992 La Spada AR, Roling DB, Harding AE, Warner CL, Spiegel R, Hausmanowa-Petrusewicz I, Yee WC, Fischbeck KH. Meiotic stability and genotype-phenotype correlation of the trinucleotide repeat in X-linked spinal and bulbar muscular atrophy. Nature Genetics. 2: 301-4. PMID 1303283 DOI: 10.1038/ng1292-301  0.84
1991 La Spada AR, Wilson EM, Lubahn DB, Harding AE, Fischbeck KH. Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature. 352: 77-9. PMID 2062380 DOI: 10.1038/352077a0  0.84
1991 Fischbeck KH, Souders D, La Spada A. A candidate gene for X-linked spinal muscular atrophy. Advances in Neurology. 56: 209-13. PMID 1853757  0.84
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