Abstract
Studying peripheral blood mononuclear cells (PBMCs) has become an important diagnostic tool in lysosomal storage diseases. Previous studies revealed that B and subclasses of T lymphocytes participate in the storage process, whereas the role of circulating monocytes was not clear. In this study, the involvement of CD14+ monocytes in lysosomal diseases was investigated. Blood samples from six patients with different lysosomal storage disorders were studied, including one with late-infantile and three with juvenile neuronal ceroid-lipofuscinoses, and two with mucopolysaccharidosis type VI. CD14+ cells were separated immunomagnetically from PBMCs and studied by light and electron microscopy. In all investigated disorders, disease-specific lysosomal storage material could be found in monocytes. The ratio of affected to non-affected cells did not differ from previously reported data on lymphocytes and their subforms in these diseases. Our data were obtained by studying a small number of different lysosomal storage disorders. Nevertheless, they suggest that lysosomal storage in the monocyte-macrophage system might also be found in other forms of lysosomal diseases.
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Received: 23 June 1996 / Revised: 20 March 1997 / Accepted: 28 March 1997
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Kieseier, B., Wisniewski, K. & Goebel, H. The monocyte-macrophage system is affected in lysosomal storage diseases: an immunoelectron microscopic study. Acta Neuropathol 94, 359–362 (1997). https://doi.org/10.1007/s004010050719
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DOI: https://doi.org/10.1007/s004010050719