Having established the high frequency of CXCR4hi neutrophils in SCA individuals, we next characterized their potential to act as effector cells in SCA, evaluating the activation of the Mac-1 beta integrin chain (CD11b) on the surface of these cells, as well as their expression of Toll-Like Receptor 4 (TLR4). The gene discussed is TLR4; the disease is autosomal dominant cerebellar ataxia.