Lissencephaly is a genetically heterogeneous disorder affecting normal development of the brain, resulting in structural and other abnormalities. It may occur as an isolated abnormality or in association with other syndromes. A comprehensive list of lissencephaly subtypes, as defined by OMIM, can be found by following the link in the "OMIM phenotypic series" section, below. A subset of these are listed in the table below, with links to more detailed reports for subtypes that have been investigated using fly models.
Lissencephaly is observed in severe forms of muscular dystrophy; a fly model has been used to investigate lissencephaly associated with DAG1, a human gene implicated in several forms of muscular dystrophy (see FBhh0000192).
[updated Jun. 2025 by FlyBase; FBrf0222196]
Lissencephaly represents a term for a spectrum of severe and rare brain malformations that result in a significant simplification (pachygyria) or even total absence (agyria) of brain convolutions. Based on the physical structure of the brain, lissencephaly can be generally divided into two distinct pathological forms: type I or classical lissencephaly, and type II or cobblestone lissencephaly (http://www.news-medical.net/health/Lissencephaly-Types.aspx; 2017.07.16).
Lissencephaly (LIS), literally meaning smooth brain, is characterized by a cerebral surface with few of the normal gyri (ridges) and sulci (fissures), and encompasses a spectrum of brain surface malformations. [from MIM:607432; 05.23.2016]
