title: Ultrastructure of human dermal mast cells in 29 different lysosomal storage diseases.
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.
authors.affiliation: Department of Pathology, Sackler School of Medicine, Tel Aviv University, Ramat Aviv, Israel.
authors.forename: I
authors.initials: I
authors.lastname: Hammel
authors.forename: J
authors.initials: J
authors.lastname: Alroy
authors.forename: V
authors.initials: V
authors.lastname: Goyal
authors.forename: S J
authors.initials: SJ
authors.lastname: Galli
mesh_terms: D001706:Biopsy; D003594:Cytoplasmic Granules / Q000648:ultrastructure; D006801:Humans; D016464:Lysosomal Storage Diseases / Q000473:pathology*; D008247:Lysosomes / Q000648:ultrastructure; D008407:Mast Cells / Q000648:ultrastructure*; D012189:Retrospective Studies; D012867:Skin / Q000473:pathology*