The cellular lesions of Farber's disease and their experimental reproduction in tissue culture.

@article{Rutsaert1977TheCL,
  title={The cellular lesions of Farber's disease and their experimental reproduction in tissue culture.},
  author={Jean Rutsaert and Marianne Tondeur and E Vamos-Hurwitz and Pierre Dustin},
  journal={Laboratory investigation; a journal of technical methods and pathology},
  year={1977},
  volume={36 5},
  pages={
          474-80
        }
}
In a case of Farber's disease, lysosomal inclusions were shown to contain lamellar, rectilinear, or curvilinear material. In tissue culture, when fibroblasts from the patient were overloaded with ceramides containing nonhydroxylated fatty acids a lysosomal accumulation of small curvilinear structures identical with those observed at autopsy were seen. These inclusions persisted for several weeks after the cells were replaced in a normal culture medium. Normal fibroblasts overloaded in the same… CONTINUE READING