The monocyte-macrophage system is affected in lysosomal storage diseases: an immunoelectron microscopic study

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ID: 113605
1970
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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.
Reference Key
kieseier1970actathe Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors B. C. Kieseier;K. E. Wisniewski;H. H. Goebel;B. C. Kieseier;K. E. Wisniewski;H. H. Goebel;
Journal acta neuropathologica
Year 1970
DOI
doi:10.1007/s004010050719
URL
https://link.springer.com/article/10.1007/s004010050719
Keywords
pathology neurosciences

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