Functional defects in human and mouse LYST (also known as Chédiak-Higashi/Beige) result in the appearance of large lysosome-related compartments with impaired secretion and increased susceptibility to infection, while loss of LYST gene causes a severe immunodeficiency such as Chédiak-Higashi syndrome [22]. Here, LYST is linked to Chediak-Higashi syndrome.