Department of Neurology

Associate Professor of Neurology

BIOGRAPHICAL SKETCH

Dr. Jun Li is an Associate Professor in Department of Neurology and Neuroscience program of Vanderbilt University. Dr. Li obtained his medical degree in 1985 from Anhui Medical University, People’s Republic of China. He attended Hahnemann University in Philadelphia, United States (present name = Drexel University College of Medicine), where he earned a PhD in Neuroscience in 1995. He completed his Neurology residency in Ohio State University in 1999 and EMG/Neuromuscular Disease fellowship training in University of Utah in 2000.

In August 2000, Dr. Li joined the faculty at Wayne State University School of Medicine as an Assistant Professor of Neurology. He was promoted to Associate Professor of Neurology in 2005. He stayed at Wayne State University until August 2009, and was recruited into Department of Neurology, Vanderbilt University as an Associate Professor in September 2009.

Dr. Li has been the recipient of David Kotlarek Award from the Department of Neurology, Ohio State University for the excellence in patient care in 1999. In 2004, he received a K08 award from NIH. Since 2005, he has been elected as Best Doctors in America. In 2005, he received a Faculty Research Excellence Award from Wayne State University School of Medicine. In 2008, he received a Junior Faculty Award – Science from Wayne State University.

Dr. Li is an editorial member for Journal of Peripheral Nervous System. He has served as a grant reviewer in the study section of Muscular Dystrophy Association and reviewers for a variety of journals in the neuromuscular field. He has been invited to give numerous talks at universities and domestic/international meetings. His research is currently funded by NIH, Muscular Dystrophy Association, and Veterans Affairs.

RESEARCH SUMMARY

Dr. Li has been interested in Inherited Neuropathies (also called Charcot-Marie-Tooth disease or CMT) and Myelin Biology. Disabilities in many neurological diseases, including inherited neuropathies and motor neuron diseases, are usually caused by one of the two pathophysiological processes: conduction block and/or axonal degeneration. My laboratory investigates the molecular mechanisms of the two processes at different levels of biological system, including primary culture neurons/Schwann cells, genetically manipulated rodent models, and human subjects with inherited neuropathies or motor neuron diseases. This approach allows us not only to understand the disease better, but also provides opportunity to observe the basic functions of specific genes and coded proteins in vitro, in vivo and human subjects.

We evaluate patients in our clinic dedicated specifically to CMT. Their genotypic and phenotypic manifestations are carefully characterized. The characterization is often assisted by non-invasive techniques, such as conventional nerve conduction study, needle EMG, motor unit number estimation (MUNE), skin biopsies, muscle MRS and neuropathy scores, etc. Data derived from these evaluations raise important scientific questions, which are further explored in vitro and in the related animal models. These are usually achieved by using knockout or transgenic rodents, primary neuronal culture, neuron/Schwann cell coculture, DNA transfection, protein chemistry, immunohistochemistry, confocal microscopy, time-lapse imaging, electron microscopy, immunoEM, animal behavior tests, etc. These translational approaches have proven to be fruitful as shown in the publications listed below.

 

SELECTED PUBLICATIONS

Original Observations in Refereed Journal:

1. Li J, Krajewski KM, Shy ME, Lewis RA, Hereditary neuropathy with liability to pressure palsy: the electrophysiology fits the name. Neurology 2002; 58:1769-73.

2. Li J, Loeb JA, Shy ME, Shah AK, Tselis AC, Kupsky WJ, Lewis RA. Asymmetric Flaccid Paralysis: A Neuromuscular Presentation of West Nile Virus Infection. Ann Neurol 2003; 53 (6): 703-710.

3. Shy ME, Jani A, Krajewski K, Lewis RA, Li J, Shy RR, Balsamo J, Lilien J, Garbern JY, Kamholz J. Phenotypic clustering in MPZ mutations. Brain 2004; 127: 371-84.

4. Hu J, Xia Y, Shen Y, Li J, Zuo CS, Xuan Y, Jiang Q. Significant differences in proton trimethyl ammonium signals between human gastrocnemius and soleus muscle. J Magn Reson Imaging 2004;19: 617-22.

5. Shy ME, Blake J, Krajewski K, Fuerst DR, Laura M, Hahn A, Li J, Lewis R, and Reilly M. Reliability and validity of the CMT Neuropathy Score as a measure of disability. Neurology 2005; 64(7):1209-14.

6. Li J, Bai YH, Ghandour K, Qin P, Grandis M, Trostinskaia A, Ianakova E, Wu XY, Schenone A, Vallat JM, Kupsky WJ, Hatfield J, Shy ME. Skin biopsies in myelin related neuropathies; bringing molecular pathology to the bedside. Brain, 2005;128 (Pt 5):1168-77.

7. Shy ME, Scavina MT, Clark A, Krajewski KM, Li J, Kamholz J, Kolodny E, Szigeti K, Richard A, Fischer RA, Saifi GM, Scherer SS and Lupski JR. T118M PMP22 Mutation Causes Partial Loss of Function and HNPP-like Neuropathy. Ann Neurol, 2006; 59(2):358-64.

8. Li J, Bai YH, Ianokova E, Grandis M, Uchwat F, Trostinskaia A, Krajewski KM, Garbern J, Kupsky WJ, Shy ME. Major Myelin Protein Gene (P0) Mutation Causes a Novel Form of Axonal Degeneration. J Comp Neurol; 2006; 498(2):252-265.

9. Sabet A, Li J, Ghandour K, Pu Q, Wu XY, Kamholz J, Shy ME and Cambi F. MPZ intronic mutation disrupts splicing and causes late onset CMT1B. Neurology 2006; 67: 1141-1146.

10. Bai YH, Ianakova E, Pu Q, Ghandour K, Levinson R, Martin JJ, Ceuterick-de Groote C, Mazanec R, Seeman P, Shy ME, and Li J. R69C Mutation in P0 Gene Alters Myelination and Ion Channel Subtypes. Arch Neurol 2006; 63: 1787 – 1794.

11. Li J, Ghandour K, Radovanovic D, Shy RR, Krajewski KM, Shy ME, Nicholson GA. Stoichiometric alteration of PMP22 protein determines the phenotype of HNPP. Arch Neurol 2007; 64 (7): 974-978.

12. Chow CY, Zhang YL, Dowling J, Jin N, Adamska M, Shiga K, Szigeta K, Shy ME, Li J, Zhang XB, Lupski JR, Weisman L, Meisler MH. Mutation of FIG4 encoding a PI(3,5)P2 phosphatase causes neurodegeneration in the pale tremor mouse and in patients with CMT type 4J. Nature; 2007; 448 (7149): 68-72.

13. Zhang XB, Zeng YS, Zhang W, Wang JM, Wu JL, Li J. Co-transplantation of Neural Stem Cells and NT-3-overexpressing Schwann Cells in Transected Spinal Cord. J Neurotrauma, 2007; 24(12): 1863-1877.

14. Zhang XB, Chow CY, Sahenk Z, Shy ME, Meisler MH, Li J. Mutation of FIG4 Causes a Rapidly Progressive, Asymmetric Neuronal Degeneration. Brain, 2008; 131(Pt 8):1990-2001.

15. Xiong Y, Zeng YS, Zeng CG, Du BL, He LM, Quan DP, Zhang W, Wang JM, Wu JL, Li Y, Li J. Synaptic Transmission of Neural Stem Cells Seeded in 3-Dimentional PLGA Scaffolds. Biomaterials 2009; 30(22):3711-22.

16. Katona I, Wu XY, Feely SME, Sottile S, Siskind C, Miller LJ, Shy ME, and Li J. PMP22 Expression in Dermal Nerve Myelin from Patients with CMT1A. Brain 2009; 132:1734-40.

17. Saporta MA, Katona I, Lewis RA, Masse S, Shy ME, and Li J. Shortened Internodal Length of Dermal Myelinated Nerve Fibers in CMT1A. Brain 2009; accepted.

Invited Reviews:

1. Kramer L, Li J, Shi PY. West Nile Virus: Highlights of Recent Advances (invited review). Lancet Neurology, 2007; 6: 171-81 (This paper states that all authors contribute equally).

2. Li J. Hypothesis of double-polarization (an invited review). J Neurol Sci; 2008; 275: 33-36.

Books and Chapters:

1. Li J, Smith AG. “Other Hereditary Neuropathies”. p411-p435; 2005. Editors: Bromberg M and Smith G. HANDBOOK FOR PERIPHERAL NEUROPATHY. Taylor & Francis Group, Boca Raton, Florida.

2. Li J. “Myotonic dystrophy” p288-289; 2004. Editors: Lynn J, Newton H, Rae-Grant. The 5-Minute Neurology Consult. Lippincott William & Wilkins, Philadelphia.

3. Li J, Lisak RP. “Pathophysiology of Myathenia Gravis” p2624-p2628; 2003. Editors: Shields TW, Locicero III J, Ponn RB, Rusch VW. General Thoracic Surgery (6th Ed). Lippincott William & Wilkins, Philadelphia.

4. Li J, Lewis RA, Shy EM. “Charcot-Marie-Tooth Disease” P676-p686; 2003. Editors: Aminoff M and Daroff RB. ENCYCLOPEDIA OF NEUROLOGICAL SCIENCE. Elsevier Science, Burlington, MA.

5. Shy ME, Kamholz J, Li J. “Mutations in Schwann cell genes causing inherited neuropathies” 2007; Editor: Armati, PJA. The Biology of Schwann Cells. Cambridge University Press, UK.

6. Kamholz J, Brucal M, Li J and Shy ME. “Myelin protein zero and CMT1B, A tale of two phenotypes” 2007; Editor: Steve Waxman. Molecular Neurology. Academic Press; Burlington, MA.