Professor and Director, Dorris Neuroscience Center
Faculty, Kellogg School of Science and Technology
Auditory Perception and Neuronal Circuit Formation: From Mouse Models to Human Genetic Disease
A fundamental unresolved question in biology is how our nervous system creates an internal representation of the external world. Sense organs convert signals such as light and sound into electrical impulses that are processed by the nervous system to create a reflection of our surroundings and to elicit appropriate behavioral responses. Selective pressures during evolution have shaped our genome and optimize sense organs and neuronal circuits for their tasks. Of all our sensory systems, the auditory system is the least well understood at the molecular level. My laboratory identifies and studies genes that control the function of the auditory sense organ of mammals. We also analyze the mechanisms that establish neuronal connections between the auditory sense organs and the cerebral cortex, and the formation of cell layers and neuronal circuits within the cortex.
Xiong, W., Grillet, N., Elledge, H., Wagner, T.E.J., Zhao, B., Johnson, K.R., Kazmierczak, P., and Müller, U. (2012) TMHS is an Integral Component of the Mechanotransduction Machienry of Cochlear Hair Cells. Cell, in press.
Franco, S.J., Gil-Sanz, C., Martinez-Garay, I., Espinosa, A., Harkins-Perry, S.R., Ramos, C., and Müller, U. (2012) . Fate-restricted neural progenitors in the mammalian cerebral cortex. Science 337:746-749.
Franco, S.J., Martinez-Garay, I., Gil-Sanz, C., Harkins-Perry, S.R., Müller, U. (2011) Reelin regulates cadherin function via Dab1/Rap1 to control neuronal migration and lamination in the neocortex. Neuron 69(3):482-97.
Gillespie, P.G., Müller, U. (2009) Mechanotransduction by hair cells: models, molecules, and mechanisms. Cell 139(1):33-44.
Grillet, N., Xiong, W., Reynolds, A., Kazmierczak, P., Sto, T., Lillo, C., Dumont, R.A., Hintermann, E., Sczaniecka, A., Schwander, M., Williams, D., Kachar, B., Gillespie, P.G., Müller, U. (2009) Harmonin mutations cause mechanotransduction defects in the cochlear hair cells. Neuron 62(3):375-87.
Kazmierczak, P.†, Sakaguchi, H. †, Tokita, J., Wilson-Kubalek, E.M., Milligan, R.A., Müller, U.*, and Kachar, B.* (2007). Cadherin 23 and protocadherin 15 interact to form tip-link filaments in sensory hair cells. Nature 449, 87-91. † co-first authors, * co-corresponding authors