- About this Journal
- Abstracting and Indexing
- Aims and Scope
- Article Processing Charges
- Articles in Press
- Author Guidelines
- Bibliographic Information
- Citations to this Journal
- Contact Information
- Editorial Board
- Editorial Workflow
- Free eTOC Alerts
- Publication Ethics
- Reviewers Acknowledgment
- Submit a Manuscript
- Subscription Information
- Table of Contents
Neurology Research International
Volume 2011 (2011), Article ID 563784, 7 pages
Huntington's Disease: An Immune Perspective
1Centre for Infection, Immunity and Disease Mechanisms, Biosciences School of Health Sciences and Social Care, Brunel University, West London UB8 3PH, UK
2Centre of Biotechnology and Bioinformatics, School of Life Sciences, Jawaharlal Nehru Institute for Advanced Study, Secunderabad, Andhra Pradesh, India
3Centre for Cellular and Molecular Biology, Uppal Road, Hyderabad 500 007, India
4Unit of Neurology, Department of Neurological Disorders, Santa Chiara Hospital, Largo Medaglie d'oro 1, 38100 Trento, Italy
Received 17 November 2010; Revised 31 March 2011; Accepted 12 May 2011
Academic Editor: Mohammed Rachidi
Copyright © 2011 Annapurna Nayak et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
- G. Huntington, “On Chorea,” Medical and Surgical Reporter of Philadelphia, vol. 26, pp. 317–321, 1872.
- F. O. Walker, “Huntington's disease,” Lancet, vol. 369, no. 9557, pp. 218–228, 2007.
- S. E. Purdon, E. Mohr, V. Ilivitsky, and B. D. Jones, “Huntington's disease: pathogenesis, diagnosis and treatment,” Journal of Psychiatry and Neuroscience, vol. 19, no. 5, pp. 359–367, 1994.
- A. L. Orr, S. Li, C. E. Wang et al., “N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking,” Journal of Neuroscience, vol. 28, no. 11, pp. 2783–2792, 2008.
- A. Reiner, I. Dragatsis, S. Zeitlin, and D. Goldowitz, “Wild-type huntingtin plays a role in brain development and neuronal survival,” Molecular Neurobiology, vol. 28, no. 3, pp. 259–275, 2003.
- M. P. Duyao, A. B. Auerbach, A. Ryan et al., “Inactivation of the mouse Huntington's disease gene homolog Hdh,” Science, vol. 269, no. 5222, pp. 407–410, 1995.
- A. Reiner, N. Del Mar, C. A. Meade et al., “Neurons lacking huntingtin differentially colonize brain and survive in chimeric mice,” Journal of Neuroscience, vol. 21, no. 19, pp. 7608–7619, 2001.
- J. B. Penney Jr., J. P. Vonsattel, M. E. MacDonald, J. F. Gusella, and R. H. Myers, “CAG repeat number governs the development rate of pathology in huntington's disease,” Annals of Neurology, vol. 41, no. 5, pp. 689–692, 1997.
- M. DiFiglia, E. Sapp, K. O. Chase et al., “Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain,” Science, vol. 277, no. 5334, pp. 1990–1993, 1997.
- D. C. Rubinsztein, “Lessons from animal models of Huntington's disease,” Trends in Genetics, vol. 18, no. 4, pp. 202–209, 2002.
- N. Pavese, A. Gerhard, Y. F. Tai et al., “Microglial activation correlates with severity in Huntington disease: a clinical and PET study,” Neurology, vol. 66, no. 11, pp. 1638–1643, 2006.
- Y. F. Tai, N. Pavese, A. Gerhard et al., “Microglial activation in presymptomatic Huntington's disease gene carriers,” Brain, vol. 130, no. 7, pp. 1759–1766, 2007.
- K. Sathasivam, C. Hobbs, M. Turmaine et al., “Formation of polyglutamine inclusions in non-CNS tissue,” Human Molecular Genetics, vol. 8, no. 5, pp. 813–822, 1999.
- R. B. Banati, “Visualising microglial activation in vivo,” GLIA, vol. 40, no. 2, pp. 206–217, 2002.
- S. U. Kim and J. de Vellis, “Microglia in health and disease,” Journal of Neuroscience Research, vol. 81, no. 3, pp. 302–313, 2005.
- W. Zhu, H. Zheng, X. Shao, W. Wang, Q. Yao, and Z. Li, “Excitotoxicity of TNFα derived from KA activated microglia on hippocampal neurons in vitro and in vivo,” Journal of Neurochemistry, vol. 114, no. 2, pp. 386–396, 2010.
- X. Wang, S. Chen, G. Ma, M. Ye, and G. Lu, “Involvement of proinflammatory factors, apoptosis, caspase-3 activation and Ca2+ disturbance in microglia activation-mediated dopaminergic cell degeneration,” Mechanisms of Ageing and Development, vol. 126, no. 12, pp. 1241–1254, 2005.
- D. M. Bonifati and U. Kishore, “Role of complement in neurodegeneration and neuroinflammation,” Molecular Immunology, vol. 44, no. 5, pp. 999–1010, 2007.
- J. P. Vonsattel, R. H. Myers, and T. J. Stevens, “Neuropathological classification of Huntington's disease,” Journal of Neuropathology and Experimental Neurology, vol. 44, no. 6, pp. 559–577, 1985.
- A. J. Milnerwood and L. A. Raymond, “Early synaptic pathophysiology in neurodegeneration: insights from Huntington's disease,” Trends in Neurosciences, vol. 33, no. 11, pp. 513–523, 2010.
- E. Sapp, K. B. Kegel, N. Aronin et al., “Early and progressive accumulation of reactive microglia in the Huntington disease brain,” Journal of Neuropathology and Experimental Neurology, vol. 60, no. 2, pp. 161–172, 2001.
- M. Björkqvist, E. J. Wild, J. Thiele et al., “A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease,” Journal of Experimental Medicine, vol. 205, no. 8, pp. 1869–1877, 2008.
- S. K. Singhrao, J. W. Neal, B. P. Morgan, and P. Gasque, “Increased complement biosynthesis by microglia and complement activation on neurons in Huntington's disease,” Experimental Neurology, vol. 159, no. 2, pp. 362–376, 1999.
- N. S. K. Haque, P. Borghesani, and O. Isacson, “Therapeutic strategies for Huntington's disease based on a molecular understanding of the disorder,” Molecular Medicine Today, vol. 3, no. 4, pp. 175–183, 1997.
- G. J. Arlaud, C. Gaboriaud, N. M. Thielens, M. Budayova-Spano, V. Rossi, and J. C. Fontecilla-Camps, “Structural biology of the C1 complex of complement unveils the mechanisms of its activation and proteolytic activity,” Molecular Immunology, vol. 39, no. 7-8, pp. 383–394, 2002.
- U. Kishore and K. B. Reid, “C1q: structure, function, and receptors,” Immunopharmacology, vol. 49, no. 1-2, pp. 159–170, 2000.
- T. Fujita, M. Matsushita, and Y. Endo, “The lectin-complement pathway—its role in innate immunity and evolution,” Immunological Reviews, vol. 198, pp. 185–202, 2004.
- H. Rus, C. Cudrici, S. David, and F. Niculescu, “The complement system in central nervous system diseases,” Autoimmunity, vol. 39, no. 5, pp. 395–402, 2006.
- B. J. C. Janssen, E. G. Huizinga, H. C. A. Raaijmakers et al., “Structures of complement component C3 provide insights into the function and evolution of immunity,” Nature, vol. 437, no. 7058, pp. 505–511, 2005.
- S. D. Webster, A. J. Yang, L. Margol, W. Garzon-Rodriguez, C. G. Glabe, and A. J. Tenner, “Complement component C1q modulates the phagocytosis of Aβ by microglia,” Experimental Neurology, vol. 161, no. 1, pp. 127–138, 2000.
- P. Vanguri and M. L. Shin, “Activation of complement by myelin: identification of C1-binding proteins of human myelin from central nervous tissue,” Journal of Neurochemistry, vol. 46, no. 5, pp. 1535–1541, 1986.
- A. Dalrymple, E. J. Wild, R. Joubert et al., “Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates,” Journal of Proteome Research, vol. 6, no. 7, pp. 2833–2840, 2007.
- M. Thambisetty, A. Simmons, L. Velayudhan et al., “Association of plasma clusterin concentration with severity, pathology, and progression in Alzheimer disease,” Archives of General Psychiatry, vol. 67, no. 7, pp. 739–748, 2010.
- Y. Du, K. R. Bales, R. C. Dodel et al., “α2-macroglobulin attenuates β-amyloid peptide 1-40 fibril formation and associated neurotoxicity of cultured fetal rat cortical neurons,” Journal of Neurochemistry, vol. 70, no. 3, pp. 1182–1188, 1998.
- T. C. Frank-Cannon, L. T. Alto, F. E. McAlpine, and M. G. Tansey, “Does neuroinflammation fan the flame in neurodegenerative diseases?” Molecular Neurodegeneration, vol. 4, no. 1, article 47, 2009.
- W. J. Streit, R. E. Mrak, and W. S. T. Griffin, “Microglia and neuroinflammation: a pathological perspective,” Journal of Neuroinflammation, vol. 1, article 14, 2004.
- Y. F. Tai, N. Pavese, A. Gerhard et al., “Imaging microglial activation in Huntington's disease,” Brain Research Bulletin, vol. 72, no. 2-3, pp. 148–151, 2007.
- G. Hoffner, S. Souès, and P. Djian, “Aggregation of expanded huntingtin in the brains of patients with Huntington disease,” Prion, vol. 1, no. 1, pp. 26–31, 2007.
- J. Y. Shin, Z. H. Fang, Z. X. Yu, C. E. Wang, S. H. Li, and X. J. Li, “Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity,” Journal of Cell Biology, vol. 171, no. 6, pp. 1001–1012, 2005.
- J. Bradford, J. Y. Shin, M. Roberts et al., “Mutant huntingtin in glial cells exacerbates neurological symptoms of huntington disease mice,” Journal of Biological Chemistry, vol. 285, no. 14, pp. 10653–10661, 2010.
- N. J. Maragakis and J. D. Rothstein, “Glutamate transporters in neurologic disease,” Archives of Neurology, vol. 58, no. 3, pp. 365–370, 2001.
- D. J. Klionsky and Y. Ohsumi, “Vacuolar import of proteins and organelles from the cytoplasm,” Annual Review of Cell and Developmental Biology, vol. 15, pp. 1–32, 1999.
- K. B. Kegel, M. Kim, E. Sapp et al., “Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy,” Journal of Neuroscience, vol. 20, no. 19, pp. 7268–7278, 2000.
- B. Ravikumar, R. Duden, and D. C. Rubinsztein, “Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy,” Human Molecular Genetics, vol. 11, no. 9, pp. 1107–1117, 2002.
- M. Martinez-Vicente, Z. Talloczy, E. Wong et al., “Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease,” Nature Neuroscience, vol. 13, no. 5, pp. 567–576, 2010.
- S. Sarkar and D. C. Rubinsztein, “Huntington's disease: degradation of mutant huntingtin by autophagy,” FEBS Journal, vol. 275, no. 17, pp. 4263–4270, 2008.