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Institute of Neuroscience, NYMU
Bing-wen Soong Professor
Laboratory of NeuroGenetics
image Bing-wen Soong   Professor( Adjunct )
bwsoong@vghtpe.org.tw
Tel : 886-2-28712121 extension 3178
Fax : 886-2-2875-7584


MD, National Defense University School of Medicine,
Ph.D. National Yang-Ming University, 1992
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Lee, Yi--chung Wang, Po-shan
 
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Shan, Ding-i
 
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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 is one direction we are seriously exploring.

Another line of research has been on the hereditary sensorimotor polyneuropathy. Similar to SCAs, we have identified around 90 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 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 Shan DE, Soong BW. Reply to: Role of SCA2 mutations in early- and late-onset dopa-responsive Parkinsonism. Ann Neurol 2002;52:258.
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 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 Wang PS, Liu RS, Yang BH, Wu CC, Soong BWy. Regional patterns of cerebral glucose metabolism in spinocerebellar ataxia type 2, 3 and 6: a voxel-based FDG-positron emission tomography analysis. J Neurol 2007. (PMID: 17468965)
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. (PMID: 17450320)
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 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 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 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 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 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 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 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 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 Lee YC, Lu YC, Chang MH, Soong BWy. 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.
   
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