Ultrastructure of human dermal mast cells in 29 different lysosomal storage diseases |
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Authors: | Ilan Hammel Joseph Alroy Vibha Goyal Stephen J Galli |
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Institution: | 1. Department of Pathology, Sackler School of Medicine, Tel Aviv University, Ramat Aviv, Tel Aviv, Israel 2. Department of Pathology, Tufts University Schools of Medicine and Veterinary Medicine, Boston, Massachusetts, USA 3. Division of Experimental Pathology, Department of Pathology, Beth Israel Hospital and Harvard Medical School, 330 Brookline Avenue, 02215, Boston, Massachusetts, USA
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Abstract: | The effect of lysosomal storage diseases on the ultrastructure of human mast cells has not previously been reported. Indeed,
there has been little published evidence indicating that mast cells contain typical lysosomes. However, mast cell cytoplasmic
granules contain hydrolases similar to those found in lysosomes, but which differ from lysosomal hydrolases in exhibiting
optimal activity at higher pH. We therefore examined by transmission electron microscopy the dermal mast cells in 58 biopsies
of patients exhibiting 1 of 29 different lysosomal storage diseases. We found mast cells containing abnormal lysosomes in
16 of these disorders. In 6 of these 16 diseases, the mast cells' cytoplasmic granules appeared normal. These observations
indicate that human mast cells can contain lysosomes, and provide evidence that the enzymes affected by lysosomal storage
diseases are active in mast cells. |
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Keywords: | Human mast cells Lysosomal storage diseases Secretory granules |
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