Defects in early developmental processes during embryonic neurogenesis are the biological rationale of several human neuropathologies. In particular, many genes, shown to play a pivotal role in key processes as neural stem cell proliferation, early neural commitment and cell migration, are responsible of human hereditary neural syndromes. We are interested to understand the genetic mechanisms that control the behavior and fate of neural stem cells and the pathways by which different types of neurons arise by a similar pool of neuroblasts. Interestingly, some transcription factors playing a crucial function in GABArgic neural differentiation have been found mutated in neural syndromes leading to epilepsy, mental retardation, West Syndrome and some forms of autism. Understanding how these genes act in directing neural differentiation and how their mutations are causing the disease is a main challenge of the current investigations pursued in the lab. These efforts are instrumental to establish in vitro protocols of neural stem cell differentiation to tightly control their specific neuronal cell type (GABAergic, Glutamatergic or Dopaminergic) and their maturation and activity state.