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Faculty Detail     Name SCOTT M WILSON   Campus Address SHEL 914 Zip 2182 Phone 205-975-5573 E-mail livvy01@uab.edu Other websites

Faculty Appointment(s) Appointment Type Department Division Rank Primary  Neurobiology  Neurobiology Associate Professor Secondary  Biochemistry & Molecular Genetics  Biochemistry & Molecular Genetics Assistant Professor Secondary  Genetics   Genetic & Translational Med Assistant Professor Center  Civitan International Research Center  Civitan International Research Center Associate Professor Center  Comprehensive Cancer Center  Comprehensive Cancer Center Associate Professor Center  General Clinical Research Center  Comprehensive Neuroscience Center Associate Professor Center  Ctr for Glial Bio in Med  Ctr for Glial Bio in Med Associate Professor

Graduate Biomedical Sciences Affiliations Biochemistry and Molecular Genetics Program  Cell, Molecular, & Developmental Biology  Cellular and Molecular Biology Program  Genetics and Genomic Sciences  Integrative Genetics Graduate Program  Medical Scientist Training Program  Neuroscience

Biographical Sketch  He is an Associate Professor in the Department of Neurobiology at the University of Alabama at Birmingham. He was an undergraduate at the University of South Florida and then went on to the University of Florida as a graduate student under the supervision of Maurice Swanson. During his studies with Dr. Swanson, he biochemically isolated the first family of heterogeneous nuclear ribonuclear proteins in yeast and performed classical genetics to investigate the function of hnRNPs in yeast. He then began his training in mouse genetics in the laboratory of Drs. Neal Copeland and Nancy Jenkins at the National Cancer Institute as a postdoctoral fellow. While at the NCI he used a positional cloning approach to identify the mouse mutations ashen, waltzer and ataxia. The identification of these gene products has allowed for important insights into vesicular trafficking, auditory function, and neurodegeneration respectively. In addition to these projects, he also studied the function of voltage gated calcium channels by generating gene knockouts for the alpha1A and alpha1E subunits in mice. He joined the faculty at UAB in 2002 to continue his studies on the nervous system using mouse models of neurodegeneration.

Research/Clinical Interest Title Mouse Models of Neurodegeneration Description The identification of genes involved in neurodegeneration is a powerful means to understand the mechanisms of neuronal cell loss.  Our laboratory focuses on identifying these genes through a variety of approaches in mice that include transgenics, gene-knockouts and positional cloning.  We have recently cloned the mouse neurological mutation ataxia.  The ataxia mouse displays a severe tremor and hind limb paralysis by 5 weeks of age.  We showed that ataxia gene encodes Usp14, a member of the ubiquitin/proteosomepathway.   During our analysis of the ataxia mouse, we found that loss of Usp14 results in synaptic transmission defects in both the central and peripherial nervous sytem.  Since the members of this pathway act on a variety of substrates, we believe that the identification of the substrate(s) for Usp14 will provide important insights into the pathogenesis of the ataxia tremor and paralysis.  In addition to Usp14, we are also investigating the function of several other members of the ubiquitin/proteosome pathway that are involved in neuronal function. The mouse waltzer mutation is another mutation that we recently cloned. The waltzer gene encodes Cdh23, the newest member of the cadherin superfamily. Loss of this gene product in humans results in both auditory and vestibular dysfunction. We are currently producing antibodies and generating other alleles of Cdh23 to understand how this gene product functions in the perception of sound and maintenance of balance.

Selected Publications  Publication PUBMEDID Jin YN, Chen PC, Watson JA, Walters BJ, Phillips SE, Green K, Schmidt R, Wilson JA, Johnson GV, Roberson ED, Dobrunz LE, Wilson SM. Usp14 Deficiency Increases Tau Phosphorylation without Altering Tau Degradation or Causing Tau-Dependent Deficits. PLoS One. 2012;7(10) Epub 2012 Oct 29.  23144711  Chen PC, Bhattacharyya BJ, Hanna J, Minkel H, Wilson JA, Finley D, Miller RJ, Wilson SM. 2011. Ubiquitin homeostasis is critical for synaptic development and function. J Neurosci.31(48):17505-13  22131412   Bhattacharyya BJ, Wilson SM, Jung H, Miller RJ. 2011. Altered neurotransmitter release machinery in mice deficient for the de-ubiquitinating enzyme, Usp14. Am J Physiol Cell Physiol. Nov 9  22075695  Lee BH, Lee MJ, Park S, Oh DC, Elsasser S, Chen PC, Gartner C, Dimova N, Hanna J, Gygi SP, Wilson SM, King RW, Finley D. 2010. Enhancement of proteasome activity by a small-molecule inhibitor of USP14. Nature 467(7312):179-84.  20829789  Lappe-Siefke C, Loebrich S, Hevers W, Waidmann OB, Schweizer M, Fehr S, Fritschy JM, Dikic I, Eilers J, Wilson SM, Kneussel M. The ataxia (axJ) mutation causes abnormal GABAA receptor turnover in mice. PLoS Genet. 2009 Sep;5(9):e1000631  19759851  Chen PC, Qin LN, Li XM, Walters BJ, Wilson JA, Mei L, Wilson SM. 2009. The proteasome-associated deubiquitinating enzyme Usp14 is essential for the maintenance of synaptic ubiquitin levels and the development of neuromuscular junctions. J Neurosci. 29(35):10909-19.  19726649  Cartier AE, Djakovic SN, Salehi A, Wilson SM, Masliah E, Patrick GN. 2009. Regulation of synaptic structure by ubiquitin C-terminal hydrolase L1.J Neurosci. 29(24):7857-68.  19535597  Qiao L, Hamamichi S, Caldwell KA, Caldwell GA, Yacoubian TA, Wilson S, Xie ZL, Speake LD, Parks R, Crabtree D, Liang Q, Crimmins S, Schneider L, Uchiyama Y, Iwatsubo T, Zhou Y, Peng L, Lu Y, Standaert DG, Walls KC, Shacka JJ, Roth KA, Zhang J. Lysosomal enzyme cathepsin D protects against alpha-synuclein aggregation and toxicity. Mol Brain. 2008 Nov 21;1:17. doi: 10.1186/1756-6606-1-17  19021916  Crimmins S, Sutovsky M, Chen PC, Huffman A, Wheeler C, Swing DA, Roth K, Wilson J, Sutovsky P, Wilson S. 2009. Transgenic rescue of ataxia mice reveals a male-specific sterility defect. Dev Biol. 325(1):33-42.  18926813  Walters BJ, Campbell SL, Chen PC, Taylor AP, Schroeder DG, Dobrunz LE, Artavanis-Tsakonas K, Ploegh HL, Wilson JA, Cox GA, Wilson SM. 2008. Differential effects of Usp14 and Uch-L1 on the ubiquitin proteasome system and synaptic activity. Mol Cell Neurosci. 39(4):539-48.  18771733  First Prev   1 2 of  2  Next Last

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