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RESEARCH PRODUCT
The monocyte-macrophage system is affected in lysosomal storage diseases: an immunoelectron microscopic study
B. C. KieseierK. E. WisniewskiHans-hilmar Goebelsubject
Mucopolysaccharidosis VIMacrophagesMucopolysaccharidosisCD14MonocyteMucopolysaccharidosis type VILipopolysaccharide ReceptorsBiologymedicine.diseasePeripheral blood mononuclear cellMonocytesPathology and Forensic MedicineLysosomal Storage DiseasesCellular and Molecular Neurosciencemedicine.anatomical_structureNeuronal Ceroid-LipofuscinosesImmunologyLysosomal storage diseasemedicineHumansMacrophageNeuronal ceroid lipofuscinosisNeurology (clinical)Microscopy Immunoelectrondescription
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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 1997-10-12 | Acta Neuropathologica |