Characterization of T-cell subclasses and NK-cells in lysosomal disorders by immuno-electron microscopy.

Abstract

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.

• Authors: B C Kieseier, H H Goebel
• Journal: Neuropathology and applied neurobiology (Neuropathol Appl Neurobiol) Vol. 20 Issue 6 Pg. 604-8 (Dec 1994) ISSN: 0305-1846 [Print] England
• PMID: 7898623 (Publication Type: Journal Article)

Topics

• CD4-Positive T-Lymphocytes (immunology)
• CD8-Positive T-Lymphocytes (immunology)
• Humans
• Immunoblotting
• Killer Cells, Natural (immunology)
• Lysosomes (immunology)
• Microscopy, Immunoelectron
• Mucopolysaccharidoses (immunology)
• T-Lymphocytes (immunology)