Characterization of T-cell subclasses and NK-cells in lysosomal disorders by immuno-electron microscopy.
Previous studies have shown that B and T lymphocytes are affected in lysosomal disorders. The aim of this study was to investigate the involvement of subclasses of T lymphocytes and natural killer cells in lysosomal diseases. CD4+, CD8+, and CD56+ cells were immunomagnetically separated from peripheral blood mononuclear cells in 10 patients with various lysosomal diseases--including one patient each with infantile, late infantile, and juvenile neuronal ceroid-lipfuscinoses, two patients with mucopolysaccharidosis (MPS) type I and four patients with MPS type III, and one patient with mucolipidosis type II; all lymphocytes were studied by light and electron microscopy. Respective vacuolar or non-vacuolar lysosomal inclusions were identified in CD4+, CD8+, and CD56+ cells. Subclass-specific abnormal lysosomes could not be found. Both subclasses of T lymphocytes investigated showed nearly the same ratio of affected to non-affected cells. These results were obtained by studying a small number of different lysosomal disorders. Nevertheless, they suggest that lysosomal storage in these subclasses of immunocompetent cells might also be found in other forms of lysosomal disease.
Kieseier
B C
BC
Division of Neuropathology, Johannes-Gutenberg-University, Mainz, Germany.
Goebel
H H
HH
D015496:CD4-Positive T-Lymphocytes / Q000276:immunology; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology; D006801:Humans; D015151:Immunoblotting; D007694:Killer Cells, Natural / Q000276:immunology*; D008247:Lysosomes / Q000276:immunology*; D016253:Microscopy, Immunoelectron; D009083:Mucopolysaccharidoses / Q000276:immunology*; D013601:T-Lymphocytes / Q000276:immunology*