JOSH BONKOWSKY
Josh Bonkowsky
email: joshua.bonkowsky@hsc.utah.edu		 Assistant Professor of Pediatrics
Adjunct Assistant Professor of Neurobiology and Anatomy
Developmental Neurobiology

B.A. 1991, Harvard College; M.D., Ph.D. 2000, University of California, San Diego, 2003-2006, Postdoctoral Fellowship, University of Utah.

RESEARCH:

Axon pathfinding and development of basal ganglia; function of language genes in CNS development

Our research is centered on studying pediatric neurodevelopmental disorders. Neurodevelopmental disorders are very common, but poorly understood in terms of their pathophysiology and effects on CNS development. To address these disorders, we are taking both a gene-specific approach, as well as a non-biased strategy. We are currently focused on two main areas:

1. We are characterizing the language gene, foxP2, and its role in CNS development and axon pathfinding. To date, FOXP2 is the only gene which has been demonstrated to have a specific effect on language development, but its role in CNS development is not known. The regulation of foxP2 has been analyzed in conjunction with the lab of Dr. Richard Dorsky, and we have found that the transcription factor lef1 is necessary for foxP2 expression in the tectum and hindbrain. We have developed transgenic zebrafish which express GFP in the foxP2 neurons, and we are now studying what effects loss of foxP2 has on the development of neural circuits, and what downstream genes mediate these effects.

2. We are studying the development of the basal ganglia and their connections. The basal ganglia play a critical role in the CNS, and dysfunction underlie many common neurological disorders (cerebral palsy, Parkinson's, and Huntington's, for example). We have developed unique transgenic lines which specifically label subsets of basal ganglia neurons, allowing us to perform live visualization of their development. These transgenic lines are allowing us to analyze which genes are necessary for the development of basal ganglia connectivity, as well as the effects of hypoxia and other toxic insults.

Selected Publications

Kastenhuber, E., Kern, U., Bonkowsky, J.L., Chien, C.-B., Driever, W., and Schweitzer, J. (2009) Netrin-DCC, Robo-Slit and HSPGs coordinate lateral positioning of longitudinal dopaminergic diencephalospinal axons. Journal of Neuroscience, 29:8914-8926.

Bonkowsky, J.L., Wang, X., Fujimoto, E., Lee, J.E., Chien, C.B., and Dorsky, R.I. (2008) Domain-specific regulation of foxP2 CNS expression by lef1. BMC Dev Biol, Oct 24; 8:103.

Bonkowsky, J.L., Filloux, F.M., and Warner, J.E. (2007) Splenial Corpus Callosum Lesion and Hemifield Visual Color Anomia Associated With Intracranial Hypertension. Journal of Child Neurology, 22:1132-1134.

Bonkowsky, J.L., and Chien, C.-B. (2005) Isolation and cloning of zebrafish foxP2. Developmental Dynamics, 234:740-746.

Bonkowsky, J.L., Bohnsack, J.F., Pennington, M.J., Viskochil, D., and Thompson, J.A. (2004) Leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia (LACH) in two brothers: a novel syndrome? American Journal of Medical Genetics, 128:52-56.

Bonkowsky, J.L., Johnson, J., Carey, J., Smith, A.D., and Swoboda, K. (2003) An infant with primary tooth loss and palmar hyperkeratosis: a novel mutation in the NTRK1 gene causing congenital insensitivity to pain with anhidrosis. Pediatrics, 112:e237-241.

Yoshikawa*, S., Bonkowsky*, J.L., Kokel, M., Shyn, S., and Thomas, J.B. (2001) The Derailed guidance receptor does not require kinase activity in vivo. Journal of Neuroscience, 21: RC119.

Bonkowsky*, J.L, Yoshikawa*, S., OÕKeefe, D.D., Scully, A.L., and Thomas, J.B. (1999) Axon routing across the midline controlled by the Drosophila Derailed receptor. Nature, 402:540-544.

Bonkowsky, J.L., and Thomas, J.B. (1998) Cell-type specific modular regulation of derailed in the Drosophila nervous system. Mechanisms of Development, 82:181-184.

Oates, A.C., Bonkowsky, J.L., Irvine, D.V., Kelly, L.E., Thomas, J.B., and Wilks, A.F. (1998) Embryonic expression and activity of doughnut, a second RYK homolog in Drosophila. Mechanisms of Development, 78:165-169.

Neuroscience Home Page Contact us Search