Regions of the frontal lobe are essential for higher cognitive function. In patients with bilateral frontoparietal polymicrogyria (BFPP), affected individuals show frontal lobe dysfunction (including mental retardation, gait difficulty, language delay, and seizures) and characteristic frontal lobe anatomy (bilaterally symmetrical, and abnormally thin with multiple small, irregular folds). Piao et al. (see Perspective by Holt and Jahn) identify the genetic basis of BFPP as mutations in a putative G protein-coupled receptor, GPR56. GPR56 appears primarily to affect neuronal progenitors and, thus, regional patterning of human cerebral cortex.
X. Piao, R. S. Hill, A. Bodell, B. S. Chang, L. Basel-Vanagaite, R. Straussberg, W. B. Dobyns, B. Qasrawi, R. M. Winter, A. M. Innes, T. Voit, M. E. Ross, J. L. Michaud, J.-C. Déscarie, A. J. Barkovich, C. A. Walsh, G protein-coupled receptor-dependent development of human frontal cortex. Science 303, 2033-2036 (2004). [Abstract] [Full Text]
P. Rakic, Genetic control of cortical convolutions. Science 303, 1983-1984 (2004). [Summary] [Full Text]
