Gaucher's cells
نویسنده
چکیده
Gaucher's cells have been extensively investigated morphologically and biochemically, and it is now thought that they represent histiocytic cells in which large amounts of glucocerebroside have accumulated because of a deficiency of ,B-glucosidase (Brady, 1972). The histiocytic nature of Gaucher's cells has been inferred from their phagocytic ability (Lee et al., 1967), high lysosomal enzyme content (Rozenszajn and Efrati, 1961), and distinctively reticuloendothelial distribution. However, this view has not been universally accepted (Roos et al., 1961; Fisher and Reidbord, 1962) and, in addition, immunological mechanisms have been invoked in the pathogenesis of the disease (Pennelli et al., 1969). Immunological surface marker studies, which have proved of great value in the characterisation of the pathological cells of a wide variety of other haematological proliferations, should therefore contribute to further understanding of the nature of the pathognomonic cells of Gaucher's disease. No previous surface-marker study of Gaucher's disease has been described, and in the present paper we report such a study in a case of the non-neuronopathic form of the disease.
منابع مشابه
Surface marker and other characteristics of Gaucher's cells.
A full surface marker study of the splenic storage cells in a case of Gaucher's disease largely substantiates the monocyte/histiocyte nature of Gaucher's cells. In addition, an apparent T-lymphocyte deficiency is demonstrated in the spleen and peripheral blood, and the possible significance of this finding is discussed.
متن کاملActivation of p38 Mitogen-Activated Protein Kinase in Gaucher’s Disease
Gaucher's disease is caused by defects in acid β-glucosidase 1 (GBA1) and has been also proposed as an inflammatory disease. GBA1 cleaves glucosylceramide to form ceramide, an established bioactive lipid, and defects in GBA1 lead to aberrant accumulation in glucosylceramide and insufficient formation of ceramide. We investigated if the pro-inflammatory kinase p38 is activated in Gaucher's disea...
متن کاملGaucher's disease of the lung causing severe pulmonary hypertension with associated acute recurrent pericarditis.
GAUCHER'S DISEASE is a familial disorder characterized by accumulation in reticuloendothelial cells of glucocerebrosides, compounds containing sphingosine, fatty acid, and glucose in equimolar amounts. The storage cells, called Gaucher cells, have a characteristic appearance, and their increasing numbers in the liver, spleen, lymph nodes, and bone marrow are responsible for most of the clinical...
متن کاملCerebroside Synthesis in Gaucher's Disease*
Familial lipodystrophic conditions, such as Gaucher's, Niemann-Pick, and Tay-Sachs disease, are characterized by the intracellular accumulation of abnormally large quantities of sphingolipids. In Gaucher's disease, the offending lipids are cerebrosides, while Niemann-Pick and Tay-Sachs diseases are characterized by the accumulation of sphingomyelin and gangliosides, respectively. In Gaucher's a...
متن کاملAcid hydrolases in leukocytes and platelets of normal subjects and in patients with Gaucher's and Fabry's disease
Lymphocytes, monocytes, neutrophilic granulocytes and platelets were each separated to greater than 95% purity from six normal subjects, three patients with Gaucher's disease, two heterozygotes for Gaucher's disease, and one patient with Fabry's disease. Activities of the following acid hydrolases were determined: "acid" (pH 4.0) beta-glucosidase, pH 5.0 beta-glucosidase, alpha-galactosidase, a...
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تاریخ انتشار 2004