Immunelectronmicroscopic characterization of T4 and T8 lymphocytes and natural killer cells in neuronal ceroid-lipofuscinosis.
CD4+, CD8+, and CD56+ cells were isolated with the immunomagnetic separation technique from peripheral blood mononuclear cells (PBMC) of 3 patients with neuronal ceroid-lipofuscinosis: one patient each with infantile (INCL), late infantile (LINCL), and juvenile (JNCL) neuronal ceroid-lipofuscinoses, all studied by light (LM) and electron (EM) microscopy. To compare the pathology of these cells with affected cells in other types of lysosomal diseases, the separation was also performed with PBMC of 1 patient with mucolipidosis (ML) type II, 2 patients with mucopolysaccharidosis (MPS) type I, and 4 patients with MPS type III. Disease-specific lysosomal inclusions were identified in CD4+, CD8+, and CD56+ cells. Subclass-specific storage products could not be found. T4 and T8 lymphocytes showed nearly the same ratio of affected to nonaffected cells. These results suggest that lysosomal storage can be found in most of the lymphocyte subclasses in these forms of neuronal ceroid-lipofuscinoses and other lysosomal diseases.
Kieseier
B C
BC
Division of Neuropathology, Johannes-Gutenberg University, Mainz, Germany.
Goebel
H H
HH
D000293:Adolescent; D015496:CD4-Positive T-Lymphocytes / Q000648:ultrastructure*; D018414:CD8-Positive T-Lymphocytes / Q000648:ultrastructure*; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007694:Killer Cells, Natural / Q000648:ultrastructure*; D008247:Lysosomes / Q000473:pathology* / Q000648:ultrastructure; D016253:Microscopy, Immunoelectron; D009083:Mucopolysaccharidoses / Q000276:immunology / Q000473:pathology; D009472:Neuronal Ceroid-Lipofuscinoses / Q000276:immunology* / Q000473:pathology