Abnormal spindle-like microcephaly-associated protein (ASPM) is a mitotic spindle protein. In Drosophila it is known as Asp, and it is required to maintain the structure of the centrosomal microtubule organising centre (MTOC) during mitosis []. In humans it is involved in spindle organisation, spindle orientation and cytokinesis []. Mutations in the ASPM gene cause autosomal recessive primary microcephaly (MCPH), which is a rare Mendelian disorder characterised by a congenital deficiency of foetal brain growth, particularly affecting the neocortex [].
The ASH (ASPM-SPD-2-Hydin) domain or N-terminal domain of abnormal spindle-like microcephaly-associated protein (ASPM) is found in proteins associated with cilia, flagella, the centrosome and the Golgi complex [, ]. The domain is also found in Hydin and OCRL whose deficiencies are associated with hydrocephalus and Lowe oculocerebrorenal syndrome (OCRL), respectively. The presence of ASH in centrosomal and ciliary proteins indicates that ASPM may possess roles not only in mitotic spindle regulation, but also in ciliary and flagellar function [].
