Robert A. Maue, Ph.D.
Professor of Physiology and Neurobiology
Professor of Biochemistry
Physiology and Neurobiology
U. California - San Diego, PHD 1985
Molecular and Cellular Biology Graduate Programs
Neuroscience Center at Dartmouth
Program in Experimental and Molecular Medicine
Geisel School of Medicine
Hanover NH 03755
Office: Remsen 328
Phone: 603-650-1311/ 650-1119 (l
We are interested in understanding the mechanisms important for the development and differentiation of neurons in the brain. In particular, we are interested in the means by which a family of growth factors known as neurotrophins (NGF, BDNF, NT-3) exert their influence the growth and electrical activity of neurons, including their regulation of process outgrowth and ion channel expression. Our recent efforts have focused on a class of neurons in the brain known as cerebellar Purkinje cells, and our interest in events associated with their development has recently focused on the pathology and abnormal development of these neurons in neurological disorders such as Niemann Pick Type C (NPC) disease. NPC disease is a fatal genetic disorder associated with abnormal cellular cholesterol accumulation, and a hallmark of this disorder is the preferential loss of Purkinje cells. Given the recent appreciation of the importance of cholesterol in the brain, particularly during neurodegenerative diseases such as NPC disease, Alzheimer's disease, and Huntington’s disease, understanding the effects of NPC disease and cholesterol abnormalities on Purkinje cells has widespread implications.
In our experiments we have taken advantage of transgenic mice expressing ion channel genes, knockout mice lacking neurotrophin receptors, and novel mouse models of NPC disease that we've developed to analyze neurons in primary culture, in brain slices, and in vivo. This has included immunocytochemical and morphometric analyses, Western blotting and biochemical assays, single-cell, real time PCR analyses of gene expression, patch clamp recording of ion channels and electrical activity, virus-mediated expression of fluorescent proteins in vivo and in cultured neurons, and behavioral analyses of cerebellar function.
Rotations and Thesis Projects:
Among future studies include 1) behavioral and histological evaluation of potential treatments for NPC disease, and 2) analysis of genes encoding ion channels, calcium binding proteins, and enyzmes involved in cholesterol metabolism in wild type and mutant Purkinje cells, using single-cell, real time PCR.
National Institutes of Health, National Niemann Pick Disease Foundation, Ara Parseghian Medical Research Foundation, Hereditary Disease Foundation, Epilepsy Foundation, Wellcome Trust
-Instructor Medical Neuroscience (Medical student course) 1990-present
-Instructor PEMM Core Course (Grad course PEMM Program) 2007-present
-Director/Lecturer Adv Biomed Sci (Grad course PEMM Program) 2008-present
-Instructor Molec Cell Biol (Grad core course - MCB Program) 1996-2005
-Instructor Receptor Pharm (Grad course -– Pharmacology Program) 1996-2006
-Director/Instructor Stystems Neuroscience course (PBS 65 upper div undergrad course) 2007-present
-Director/Instruct Cellular/ Molec Neuro course (PBS 46 upper div undergrad course) 2007-present
-Director/Instruct Physiol (Bio 14 upper division undergrad course) 2008- present
-Director/Instruct Physiology (Bio 35 upper division undergrad course) 2006
-Director/Instruct Adv Neurobiology (Bio 74 upper div undergrad course) 2006
-Instructor Biochemistry (Bio 78 upper division undergrad course) 1998-2005
-Instructor Intro Cell Biology (Bio 15 upper division undergrad course) 2003
Born in Anoka, Minnesota, a small town (now suburb) just north of Minneapolis/St Paul. After high school at Anoka Senior High School (Go Tornadoes!), went to small, all male, liberal arts college/monastery in northern Minnesota called St. John’s University and majored in Biology. Took a year off before going to graduate school at University of California at San Diego in Physiology /Pharmacology. Obtained PhD in Physiology and Pharmacology there, but not before serving as a member of two research expeditions to Antarctica. Did short Postdoctoral fellowship at Tufts-New England Medical Center in Boston before becoming an Assistant Professor at Dartmouth in 1989. Is married (to Dr. Leslie Henderson) and has two sons who have graduated from college.
Paul CA, Boegle AK, Maue RA. Before the loss: neuronal dysfunction in Niemann-Pick Type C disease. Biochim Biophys Acta. 2004 Oct 11;1685(1-3):63-76. Review. (view details on MedLine)
Paul CA, Reid PC, Boegle AK, Karten B, Zhang M, Jiang ZG, Franz D, Lin L, Chang TY, Vance JE, Blanchette-Mackie J, Maue RA. Adenovirus expressing an NPC1-GFP fusion gene corrects neuronal and nonneuronal defects associated with Niemann pick type C disease. J Neurosci Res. 2005 Sep 1;81(5):706-19. (view details on MedLine)
Fry M, Boegle AK, Maue RA. Differentiated pattern of sodium channel expression in dissociated Purkinje neurons maintained in long-term culture. J Neurochem. 2007 May;101(3):737-48. (view details on MedLine)
Maue RA. Understanding ion channel biology using epitope tags: progress, pitfalls, and promise. J Cell Physiol. 2007 Dec;213(3):618-25. Review. (view details on MedLine)
Claudepierre T, Paques M, Simonutti M, Buard I, Sahel J, Maue RA, Picaud S, Pfrieger FW. Lack of Niemann-Pick type C1 induces age-related degeneration in the mouse retina. Mol Cell Neurosci. 2010 Jan;43(1):164-76. (view details on MedLine)
Maue RA, Burgess RW, Wang B, Wooley CM, Seburn KL, Vanier MT, Rogers MA, Chang CC, Chang TY, Harris BT, Graber DJ, Penatti CA, Porter DM, Szwergold BS, Henderson LP, Totenhagen JW, Trouard TP, Borbon IA, Erickson RP. A novel mouse model of Niemann-Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations. Hum Mol Genet. 2012 Feb 15;21(4):730-50. (view details on MedLine)