编者按：这是来源于science最新一期的有关神经系统疾病的研究专辑，尤其是有关退行性疾病的最新发现，非常值得阅读！
This special issue of Science focuses on a wide range of brain diseases. The past years have seen tremendous progress in the battle against these diseases. Most promising in the long run, our understanding of the basic mechanisms underlying many of these diseases has grown. There have also been improvements in diagnostics and important additions to our arsenal of treatments and interventions. Our contributors describe some of the most exciting recent developments in the field and give a sense of the prospects for future prevention and therapy.
Aguzzi and Haass (p. 814) review the common features and the differences in the pathogenesis of Alzheimer's disease and prion disorders, both of which involve the accumulation of misfolded proteins. Dawson and Dawson (p. 819) overview the present state of our knowledge of Parkinson's disease. Their main focus is on recent progress in the unraveling of the molecular pathways that cause neurodegeneration in this disease. Kennedy et al. (p. 822) look at the complexities of the genetics underlying many neurodegenerative and neuropsychiatric disorders, such as schizophrenia or frontotemporal dementia. Zoghbi (p. 826) reviews recent developments in our understanding of autism and Rett syndrome, a developmental disorder in young children, and proposes a new model to explain the relation between these two diseases. DeKosky and Marek (p. 830) review the different imaging, biochemical, and clinical measures presently available for early detection of neurodegenerative disorders. This may trigger interventions, some of which slow the course of disease, before a large part of the damage has been done. Monsonego and Weiner (p. 834) describe the promising concept of immunotherapeutic approaches to treat Alzheimer's disease.
The News section focuses on mental illness. Psychiatrists are contemplating a major overhaul of the manual they use to define brain diseases (Helmuth, p. 808). One of the most problematic diagnoses is depression. While researchers explore possible causes of depression and associated brain dysfunction, several treatment options are wending their way through clinical trials (Holden, p. 810).
At Science's Signal Transduction Knowledge Environment (STKE, www.stke.org), Manji et al. discuss a possible role of the phosphatase calcineurin in schizophrenia; Robinson reviews contributions of neurotransmitter transporters in psychiatric disorders; and MacDonald examines evidence for the signaling functions of huntingtin, a protein altered in Huntington's disease.
In the Science of Aging Knowledge Environment (SAGE KE, www.sageke.org), Groves et al. present the case of a 48-year-old man who suffered from Huntington's disease. The case study includes pathology slides and a movie of the patient's neurological testing. An updated introduction to Alzheimer's disease by Helmuth covers diagnosis, pathology, prevention, and treatment.
Related articles in Science:
- In Sickness or in Health?
- Laura Helmuth
Science 2003 302: 808-810. (in News)[Summary] [Full Text] - Games Played by Rogue Proteins in Prion Disorders and Alzheimer's Disease
- Adriano Aguzzi and Christian Haass
Science 2003 302: 814-818. (in Review)[Abstract] [Full Text] - Molecular Pathways of Neurodegeneration in Parkinson's Disease
- Ted M. Dawson and Valina L. Dawson
Science 2003 302: 819-822. (in Review)[Abstract] [Full Text] - The Genetics of Adult-Onset Neuropsychiatric Disease: Complexities and Conundra?
- James L. Kennedy, Lindsay A. Farrer, Nancy C. Andreasen, Richard Mayeux, and Peter St George-Hyslop
Science 2003 302: 822-826. (in Review)[Abstract] [Full Text] - Postnatal Neurodevelopmental Disorders: Meeting at the Synapse?
- Huda Y. Zoghbi
Science 2003 302: 826-830. (in Viewpoint)[Abstract] [Full Text] - Looking Backward to Move Forward: Early Detection of Neurodegenerative Disorders
- Steven T. DeKosky and Kenneth Marek
Science 2003 302: 830-834. (in Review)[Abstract] [Full Text] - Immunotherapeutic Approaches to Alzheimer's Disease
- Alon Monsonego and Howard L. Weiner
Science 2003 302: 834-838. (in Review)[Abstract] [Full Text] - MEDICINE:
In a First, Infected Mice Recover From Prion Disease
- Jennifer Couzin
Science 2003 302: 763-765. (in News of the Week)[Summary] [Full Text] 
-Synuclein Locus Triplication Causes Parkinson's Disease
- A. B. Singleton, M. Farrer, J. Johnson, A. Singleton, S. Hague, J. Kachergus, M. Hulihan, T. Peuralinna, A. Dutra, R. Nussbaum, S. Lincoln, A. Crawley, M. Hanson, D. Maraganore, C. Adler, M. R. Cookson, M. Muenter, M. Baptista, D. Miller, J. Blancato, J. Hardy, and K. Gwinn-Hardy
Science 2003 302: 841. (in Brevia)[Full Text] - Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis
- Giovanna Mallucci, Andrew Dickinson, Jacqueline Linehan, Peter-Christian Klöhn, Sebastian Brandner, and John Collinge
Science 2003 302: 871-874. (in Reports)[Abstract] [Full Text] - Derepression of BDNF Transcription Involves Calcium-Dependent Phosphorylation of MeCP2
- Wen G. Chen, Qiang Chang, Yingxi Lin, Alexander Meissner, Anne E. West, Eric C. Griffith, Rudolf Jaenisch, and Michael E. Greenberg
Science 2003 302: 885-889. (in Reports)[Abstract] [Full Text] - DNA Methylation-Related Chromatin Remodeling in Activity-Dependent Bdnf Gene Regulation
- Keri Martinowich, Daisuke Hattori, Hao Wu, Shaun Fouse, Fei He, Yan Hu, Guoping Fan, and Yi E. Sun
Science 2003 302: 890-893. (in Reports)[Abstract] [Full Text]
