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國立陽明大學神經科學研究所
從分子到認知
宋秉文 教授
遺傳性神經疾病研究室
image 宋秉文  教授( 兼任 )
bwsoong@vghtpe.gov.tw
Tel : 886-2-28712121 extension 3178
Fax :
886-2-2874-8631

Ph.D., Institute of Clinical Medicine, National Yang-Ming University, 1992
MD, National Defense University School of Medicine, 1978

>> 相關聯結 :http://www.ym.edu.tw/neu/files/Soong/index.html
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王柏山 李宜中 林泳程 林恭平 林嬿婷 徐宗暐 陳倩
黃尚堅 楊文婷 鄒瑋玲 盧怡君
 
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Henry L. Paulson Joanna Jen Kenneth H. Fischbeck
Stefan M. Pulst 吳秀美 陳儀莊
陳瓊美 楊永正 劉仁賢
劉青山 蔡亭芬 蕭廣仁
鍾明怡 蘇宗笙
 
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image The Neurogenetics Laboratory has focused on the molecular diagnosis, studies of molecular mechanisms and development of potential therapies of neurological disease and diseases of the brain.

One line of our research focuses on cerebellar ataxias. We have helped molecularly identified more than 200 Chinese families in Taiwan with dominantly transmitted spinocerebellar ataxias (SCA), studied their clinical phenotypes, characterized them with neuroimaging tools including magnetic resonance imaging, positron emission tomography and magnetoencephalography, and conducted therapeutic trials. Furthermore, we have identified a novel locus, SCA22, of the spinocerebellar ataxias. We are cloning the gene responsible for the phenotype. We are also studying other trinucleotide repeat expansion diseases (Huntington’s disease, spinobulbar muscular atrophy, myotonic dystrophy). We are addressing the question whether there is a treatment for the polyglutamine repeat expansion diseases soon. RNA interference and cell therapy are two territories we are seriously and actively exploring.

Another line of research has been on the hereditary sensorimotor polyneuropathy. Similar to SCAs, we have identified more than 100 families with Charcot-Marie-Tooth disease type1, type 2 or type 3 mutations in PMP22, P0, connexin 32 genes. Morphological studies have been conducted with the electronic microscopy and immunohistochemistry techniques. We have transfected plasmids with the mutant genes into cells and are characterizing the expression patterns of the proteins.

These lines of research help us understand the molecular mechanisms of human neurological diseases in which genetic mutations cause Purkinje cell loss and defects of motor and sensory nerve functions. We are aiming to translate the research findings into clinical application in the near future.



image P.S. Recently, our research interest has also expanded to other neurogenetic diseases, including hereditary spastic paraplegias (HSP), cerebral autosomal dominant arteriopathy with subcortical ischemic leukoencephalopathy (CADASIL), and diseases caused by ion channelopathies.
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image 新型脊髓小腦萎縮症基因座之鑑定(2)
image 新型脊髓小腦萎縮症基因座之鑑定(1)
image 第二十二型脊髓小腦萎縮症之蛋白質研究
image 脊髓小腦萎縮症之分子遺傳學研究
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image Wang PS, Liu RS, Yang BH, Wu CC, Soong BW. Differences of cerebral glucose metabolism in spinocerebellar ataxia type 2, 3 and 6: a voxel-based morphometry analysis. J Neurol 2007 April 30.
image Wang PS, Liu RS, Yang BH, Soong BWy. Topographic brain mapping of the international cooperative ataxia rating scale: A positron emission tomography study. J Neurology 2007, April 22.
image Chiang MC, Chen HM, Lee YH, Chang HH, Wu YC, Soong BW, Chen CM, Wu YR, Liu CS, Niu DM, Wu JY, Chen YC, and Chern YJ. Dysregulation of C/EBPα by mutant Huntingtin causes the urea cycle deficiency in Huntington’s disease. Hum Mol Genet 2007;16:483-98.
image Lee YC, Lu YC, Chang MH, Soong BW Common mitochondrial DNA and POLG1 mutations are rare in the Chinese patient with adult-onset ataxia on Taiwan. J Neurol Sci 2007;254:65-8.
image Lee YC, Yang AH, Liu HC, Wong WJ, Lu YC, Chang MH, Soong BW. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy due to two novel mutations in the NOTCH3 gene. J Neurol Sci 2006;246:111-5.
image Hu HH, Teng MM, Hsu LC, Wong WJ, Wang LM, Luk YO, Chern CM, Soong BW, Sheng WY. A Pilot Study of a New Thrombolytic Agent for Acute Ischemic Stroke in Taiwan Within A Five-Hour Window. Stroke 2006;37:918-9.
image Chen CM, Lane HY, Wu YR, Ro LS, Chen FL, Hung WL, Hou YT, Lin CY, Huang SY, Chen IC, Soong BW, Li ML, Hsieh HM, Su MT, Lee-Chen GJ. Expanded trinucleotide repeats in the TBP/SCA17 gene, but not the KLHL1AS/SCA8 and PPP2R2B/SCA12 genes, are associated with schizophrenia. Schizophr Res 2005;78:131-6.
image Lee YC, Soong BW, Liu YT, Lin KP, Kao KP, Wu ZA. Median nerve motor conduction velocity is concordant with myelin protein zero gene mutations. J Neurol 2005;252:151-155.
image Bruno MK, Hallett M, Gwinn-Hardy K, Pugh A, Gesse M, Considine E, Tucker S, Lynch DR, Mathews KD, Swoboda KJ, Harris J, Soong BW, Ashizawa T, Dambrosia J, Jankovic J, Renner D, Fu YH, Ptacek L. Clinical evaluation of idiopathic kinesiogenic dyskinesia: new diagnostic criteria. Neurology 2004;63:2280-7.
image Shan DE, Soong BW. Presence of Spinocerebellar Ataxia Type 2 Gene Mutation in a Patient with Apparently Sporadic Parkinson’s Disease – Clinical Implications. Mov Disord 2004;19:1357-60.
image Lee YC, Soong BW, Lin KP, Lee HY, Wu ZA, Kao KP. Myelin protein zero gene mutations in Taiwanese patients with Charcot-Marie-Tooth disease type 1. J Neurol Sci 2004;219:95-100.
image Chung MY, Lu YC, Cheng NC, Soong BW. A novel autosomal dominant spinocerebellar ataxia (SCA22) linked to chromosome 1p21-q23. Brain 2003;126:1293-9.
image Lee YC, Chen JT, Liao KK, Wu ZA, Soong BW. Prolonged cortical relay time and central motor conduction in patients with spinocerebellar ataxia type 6. Clin Neurophysiol 2003;114:458-62.
image Shan DE, Soong BW. Reply to: Role of SCA2 mutations in early- and late-onset dopa-responsive Parkinsonism. Ann Neurol 2002;52:258.
image Lo HS, Hogan EL , Soong BW. 5’-Flanking region polymorphism of the neuronal nitric oxide synthase gene with Parkinson’s disease in Taiwan. J Neurol Sci 2002;194:11-3.
image Shan DE, Soong BW, Sun CM, Lee SJ, Liao KK, Liu RS. Spinocerebellar ataxia type 2 presenting as familial levodopa-responsive parkinsonism. Ann Neurol 2001;6:812-5.
image Lee IH, Soong BW, Lu YC, Chang YC. Dentatorubral-pallidoluysian Atrophy in Chinese. Arch Neurol 2001;58:1905-8.
image Soong, BW, Lu YC, Choo KB, Lee HY. Frequency analysis of autosomal dominant cerebellar ataxias in Taiwanese patients and clinical and molecular characterization of spinocerebellar ataxia type 6. Arch Neurol 2001:58:1105-9.
image Gaspar C, Lopes-Cendes I, Hayes S, Goto J, Arvidssonn K, Dias A, Silveira I, Maciel P, Coutinho P, Lima M, Zhou Y-X.,. Soong B-W, Watanabe M, Giunti P, Stevanin G, Riess O, Sasaki H, Hsieh M, Nicholson GA, Brunt E, Higgins JJ, Lauritzen M, Tranebjaerg L, Volpini V, Wood N, Ranum L, Tsuji S, Brice A, Sequeiros J, Rouleau1GA. Ancestral origins of the Machado-Joseph disease mutation: a worldwide haplotype study. Am J Hum Genet. 2001;68: 523-8.
image Soong BW, Liu RS, Wu LC, Lu YC, Lee HY. Metabolic characterization of spinocerebellar ataxia type 6. Arch Neurol 2001:58: 300-4.
image Swoboda KJ, Soong BW, McKenna C, Brunt ERP, Litt M, Bale J, Ashizawa T, Bennett LB, Bowcock AM, Roach ES, Gerson E, Matsuura T, Heydemann PT, Nespeca MP, Jankovic J, Leppert M, and Ptáček LJ. Paroxysmal Kinesiogenic dyskinesia and infantile convulsions: clinical and linkage data in eleven families. Neurology 2000;55:224-30.
image Labuda M, Labuda D, Poirier J, Soong, BW, Barucha NE, Pandolfo M. The Friedreich ataxia GAA TTC repeat: an African origin for long normal alleles and an Indo-European origin for expansions? Neurology 2000;54:2322-4.
image Soong BW, Chung MY, Lin MW. Genome-wide scanning for new loci of autosomal dominant spinocerebellar ataxias, invited lecture at the 2nd Chinese Neurologists Meeting and the 14th Hong Kong Neurologists Meeting, Hong Kong, November 11, 2001.
image Soong BW. Autosomal dominant spinocerebellar ataxias: clinical features and genetics. The invited speaker at the Grand Round, Department of Internal Medicine, East Carolina University School of Medicine. Greenville, North Carolina, USA, April 26, 2002.
image Soong BW. Spinocerebellar ataxia type 22: a novel locus of autosomal dominant spinocerebellar ataxia. The invited speaker at the 2nd International Symposium on hereditary ataxias. Holguin, Cuba, November 14, 2002.
image Soong BW. Autosomal dominant spinocerebellar ataxias: clinical features and genetics. The invited speaker at the “Emerging Trends in Chemical Pathology” symposium, COC Pathology Commissioned Training Programme 2004, Hong Kong, February 7th, 2004.
image Soong BW. An overview of Myotonic dystrophy. The invited speaker at the 4th Annual Scientific Meeting of Asian and Oceanian Myology Center, Kaohsiung, Taiwan, March 3-4, 2005.
image Soong BW. Differences of cerebral glucose metabolism in spinocerebellar ataxia type 2, 3 and 6: a voxel-based morphometry analysis. The XVIIIth World Congress of Neurology, Sydney, Australia, Nov. 8, 2005.
   
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