Neutrophil cytoskeletal disease

Neutrophils and other phagocytes migrate to the site of infection, ingest p athogens, and destroy them after releasing granule contents and active oxyg en. These activities of the cells are closely associated with a rapid reorg anization of the cytoskeleton, in which actin polymerizes, cross-links, anc hors to the membrane and depolymerizes under the control of various actin-a ssociated proteins. Defect in actin or its associated proteins results in n eutrophil cytoskeletal disease where abnormality primarily appears as motil ity or chemotactic defect of the cells. Although their molecular mechanisms have not been elucidated, neutrophil actin dysfunction and neutrophil acti n dysfunction with abnormal 47- and 89-kd proteins have been reported. Rece ntly, abnormal-beta -actin disease and disease with Rac 2 mutation, both of which accompany neutrophil chemotactic dysfunction, were analyzed at the m olecular level. These diseases are systemic, but neutrophil dysfunction of the patients is remarkable. Here we review the literature on diseases due t o cytoskeletal abnormality. Many other diseases with actin or actin-associa ted protein dysfunction may be reported in the near future. (C) 2001 The Ja panese Society of Hematology.