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Pathology of the Gaucher cell
Progress in Clinical and Biological Research
|January 1, 1982
Summary
Gaucher cells contain unique tubule-like structures due to glucocerebroside accumulation. Monocytes in Gaucher
Area of Science:
- Cell Biology
- Biochemistry
- Pathology
Background:
- Gaucher cells arise from excessive glucocerebroside accumulation in the monocyte-macrophage system.
- Gaucher cells exhibit characteristic cytoplasmic inclusions with tubule-like structures.
Purpose of the Study:
- To characterize the ultrastructure and composition of inclusions within Gaucher cells.
- To investigate the role of monocytes as precursors to Gaucher cells.
Main Methods:
- Ultrastructural analysis (freeze fracture, x-ray diffraction) of Gaucher cells.
- Acid phosphatase isoenzyme analysis.
- Morphological examination of bone marrow monocytes from Gaucher patients.
Main Results:
- Gaucher cell inclusions contain tubule-like structures (130-150 Å diameter), appearing as 6 nm thick bilayers.
- Gaucher cells possess tartaric acid-resistant acid phosphatase isoenzyme 5.
- Monocytes from Gaucher patients contain similar acid phosphatase-positive inclusions with tubule-like structures.
Conclusions:
- The ultrastructural findings provide detailed insights into Gaucher cell pathology.
- Monocytes with specific inclusions are identified as potential precursors to Gaucher cells.
- This study enhances understanding of Gaucher disease pathogenesis at a cellular level.