Jun Li
Last active: 5/20/2014

Profile

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.

Publications

The following timeline graph is generated from all co-authored publications.

Featured publications are shown below:

  1. Abnormal junctions and permeability of myelin in PMP22-deficient nerves. Guo J, Wang L, Zhang Y, Wu J, Arpag S, Hu B, Imhof BA, Tian X, Carter BD, Suter U, Li J (2014) Ann Neurol 75(2): 255-65
    › Primary publication · 24339129 (PubMed) · PMC4206215 (PubMed Central)
  2. Structure and expression of a novel compact myelin protein - small VCP-interacting protein (SVIP). Wu J, Peng D, Voehler M, Sanders CR, Li J (2013) Biochem Biophys Res Commun 440(1): 173-8
    › Primary publication · 24055875 (PubMed) · PMC3828079 (PubMed Central)
  3. Evaluation of dermal myelinated nerve fibers in diabetes mellitus. Peltier AC, Myers MI, Artibee KJ, Hamilton AD, Yan Q, Guo J, Shi Y, Wang L, Li J (2013) J Peripher Nerv Syst 18(2): 162-7
    › Primary publication · 23781963 (PubMed) · PMC3693761 (PubMed Central)
  4. Fig4 expression in the rodent nervous system and its potential role in preventing abnormal lysosomal accumulation. Guo J, Ma YH, Yan Q, Wang L, Zeng YS, Wu JL, Li J (2012) J Neuropathol Exp Neurol 71(1): 28-39
    › Primary publication · 22157617 (PubMed)
  5. Trauma does not accelerate neuronal degeneration in Fig4 insufficient mice. Yan Q, Guo J, Zhang X, Bai Y, Wang L, Li J (2012) J Neurol Sci 312(1-2): 102-7
    › Primary publication · 21872275 (PubMed)