Age-related accumulation of ceroid-like pigment in mice with Chediak-Higashi syndrome.

The Chediak-Higashi Syndrome (CHS) is a rare inherited disorder occurring in man and several animal homologs including the beige mouse; it is characterized by pigmentary dilution, susceptibility to pyogenic infections, and the presence of enlarged lysosomes in many cell types. Beige mice 6 months of age and older were found to have darkened livers, kidneys, and spleens, accompanied by splenomegaly. A fluorescence microscopic survey of tissues from beige mice revealed marked accumulations of a microscopic survey of tissues from beige mice revealed marked accumulations of a yellow autofluorescent pigment inhepatocytes, renal proximal tubule cells, and splenic macrophages.