Edward A. Doisy Department of
Biochemistry and Molecular Biology

Secondary Faculty

M.D., 1984, Saint Louis University School of Medicine
Research Interests
Board certified in pediatrics and neonatal-perinatal medicine with a clinical specialty in iron homeostasis.
Recent Publications
  • Increased hepcidin in transferrin-treated thalassemic mice correlates with increased liver BMP2 expression and decreased hepatocyte ERK activation.
    Chen H, Choosing T, et al. Haematologica. (2016) 101(3):297-308.
  • Effects of strain and age on hepatic gene expression profiles in murine models of HFE-associated hereditary hemochromatosis.
    Lee SM, Loguinov A, et al. Genes Nutr. (2015) 10(1):443.
Ph.D., 1990, Lomonosov Moscow State University
Research Interests
We are interested in the universal mechanism of transmembrane signal transduction mediated by heterotrimeric G-proteins, which are responsible for the transmission of information about extracellular messengers such as hormones, neurotransmitters and sensory stimuli, including visual signals.

Our laboratory is working on questions of the molecular organization of the receptor-G-protein complex, the high-resolution picture of the receptor-G-protein interface, the mechanism of signal transfer from the receptor, and the roles of individual G-protein subunits, especially the G-protein beta-gamma subunit complex, in this dynamic process.

The phototransduction cascade in highly specialized sensory neurons, retinal photoreceptor cells, is the prototypical G-protein-mediated system. As alterations of function of this system due to mutations, age-related processes or drug interactions may lead to severe visual disorders, one of our goals is the dissection of the phototransduction mechanism for clinical and therapeutic applications.
Recent Publications
Ph.D., 2001, Gifu University School of Medicine
Research Interests
We are working on developing a registry and growth charts for patients with MPS IVA and are in pre-clinical trials for enzyme replacement therapy for patients affected by MPS IVA. We are also developing a bone targeting system for treatment of MPS disorders, substrate reudction therapy for MPSs, and gene therapy for MPS IVA. Other areas of interest are characterization of hyaluronidases, the molecular evolution of glycosaminoglycans and peptidoglycan receptor proteins, and chondroitin sulfate metabolism.
Recent Publications
  • Clinical course of sly syndrome.
    Montano AM, Lock-Hock N, et al. J Med Genet. (2016) Feb 23 [Epub ahead of print].
  • Impact of enzyme replacement therapy and hematopoietic stem cell transplantation in patients with Morquio A syndrome.
    Tomatsu S, Sawamoto K, et al. Drugs Des Devel Ther. (2015) 9:1937-1953.
  • Di-sulfated keratan sulfate as a novel biomarker for mucopolysaccharidosis II, IVA, and IVB.
    Shimada T, Tomatsu S, et al. JIMD Rep. (2015) 21:1-13.
  • Therapies for the bone in mucopolysaccharidoses.
    Tomatsu S, Almeciga-Diaz CJ, et al. Mol Genet Metab. (2015) 114(2):94-109.
  • Activities of daily living in patients with Hunter syndrome: Impact of enzyme replacement therapy and hematopoietic stem cell transplantation.
    Tanjuakio J, Suzuki Y, et al. Mol Genet Metab. (2015) 114(2):161-169.
M.D.C.M., 1984, McGill University Faculty of Medicine
Research Interests
Our lab focuses on understanding the molecular genetic basis of mammalian kidney development and on how disruption of specific pathways leads to abnormal development of this vital organ. We use the mouse as our model system.
Recent Publications
  • Notch-Tnf signaling is required for development and homeostasis of arterial valves.
    Wang y, Wu B, et al. Eur Heart J. (2015) Oct 21 [Epub ahead of print].
  • A mouse model of Townes-Brocks syndrome expressing a truncated mutant Sall1 protein is protected from acute kidney injury.
    Hursch S, El-Achkar T, et al. Am J Physiol Renal Physiol. (2015) 309(10):F852-863.
  • Acute kidney injury after cardiac surgery: Is minocycline protective?.
    Golestaneh L, Lindsey K, et al. J Nephrol. (2015) 28(2):193-199.
  • The nucleosome remodeling and deacetylase complex in development and disease.
    Basta J and Rauchman M. Transl Res. (2015) 165(1):36-47.
M.D., 1989, Washington University School of Medicine
Research Interests
Clinical specialty in the areas of pediatric liver disease and general gastroenterology.
Recent Publications
M.D., Ph.D., 1992, Shanghai Second Medical University, China
Research Interests
We are interested in the molecular and genetic mechanisms of cholesterol homeostasis and the pathophysiology of cholesterol-related diseases. Our current research focuses on the pathophysiology of cholesterol gallstone disease and genetic analysis of gallstone (Lith) genes, the molecular physiology and genetics of the intestinal cholesterol and fatty acid absorption, the biochemistry of bile formation, and the physical-chemistry of cholesterol crystallization in bile. Also, our research is directed principally at a specific area that appears to be fundamental to understanding nonalcoholic fatty liver disease, insulin resistance, and the metabolic syndrome.
Recent Publications
  • The dangerous link between childhood and adulthood predictors of obesity and metabolic syndrome.
    Faience MF, Wang DQ, et al. Intern Emerg Med. (2016) Jan 12 [Epub ahead of print].
  • The cholecystokinin-1 receptor antagonist devazepide increases cholesterol cholelithogenesis in mice.
    Wang HH, Portincasa P, Wang DQ. Eur J Clin Invest. (2016) 46(2):158-169.
  • Lack of endogenous cholecystokinin promotes cholelithogenesis in mice.
    Wang HH, Liu M, et al. Neurogastroenterol Motil. (2016) 28(3):364-375.
  • Apolipoprotein A-V is present in bile and its secretion increases with lipid absorption in Sprague-Dawley rats.
    Zhang LS, Sato H, et al. Am J Physiol Gastrointest Liver Physiol. (2015) 309(11):G918-925.
  • Gut vagal afferents are necessary for the eating-suppressive effect of intraperitoneally administered ginsenoside Rb1 in rats.
    Shen L, Wang DQ, et al. Physical Behav. (2015) 152(Pt A):62-67.
  • Ginsenoside Rb1 increases insulin sensitivity by activating AMP-activated protein kinase in male rats.
    Shen L, Haas M, et al. Physiol Rep. (2015) 3(9):e12543.
  • The deletion of the estrogen receptor α gene reduces susceptibility to estrogen-induced cholesterol cholelithiasis in female mice.
    de Bari O, Wang HH, et al. Biochim Biophys Acta. (2015) 1852(10 Pt A):2161-2169.
  • Estrogen induces two distinct cholesterol crystallization pathways by activating ERα and GPR30 in female mice.
    de Bari O, Wang TY, et al. J Lipid Res. (2015) 56(9):1691-1700.
  • Endogenous elevation of plasma cholecystokinin does not prevent gallstones.
    Shahid RA, Wang DQ, et al. Eur J Clin Invest. (2015) 45(3):237-246.
  • A novel therapeutic effect of statins on nephrogenic diabetes insipidus.
    Bonfrate L, Porcine G, et al. J Cell Mol Med. (2015) 19(2):265-282.