Identification of Arx targets unveils new candidates for controlling cortical interneuron migration and differentiation.

ABSTRACT: Mutations in the homeobox transcription factor ARX have been found to be responsible for a wide spectrum of disorders extending from phenotypes with severe neuronal migration defects, such as lissencephaly, to mild forms of intellectual disabilities without apparent brain abnormalities, but with associated features of dystonia and epilepsy. Arx expression is mainly restricted to populations of GABA-containing neurons. Studies of the effects of ARX loss of function, either in humans or mutant mice, revealed varying defects, suggesting multiple roles of this gene in brain patterning, neuronal proliferation and migration, cell maturation and differentiation, as well as axonal outgrowth and connectivity. However, to date, little is known about how Arx functions as a transcription factor or whi

SUBMITTER: Friocourt G 

PROVIDER: S-EPMC3280452 | biostudies-literature | 2011

REPOSITORIES: biostudies-literature