Morphological and functional analysis of beige (Chèdiak-Higashi syndrome) mouse mast cells with giant granules
| Autor: | Muhammad Novrizal Abdi Sahid, Takeshi Kiyoi, Masachika Shudou, Masahito Ogasawara, Masaki Mogi, Kazutaka Maeyama, Shuang Liu |
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| Rok vydání: | 2019 |
| Předmět: |
0301 basic medicine
Cell type Neutrophils Immunology Cell Vesicular Transport Proteins Cytoplasmic Granules Cell Degranulation Mice 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine In vivo Lysosome medicine Animals Humans Immunology and Allergy Mast Cells Cells Cultured Pharmacology Chédiak–Higashi syndrome Homozygote Intracellular Signaling Peptides and Proteins Degranulation Proteins medicine.disease Molecular biology Mice Mutant Strains In vitro Killer Cells Natural Mice Inbred C57BL Disease Models Animal 030104 developmental biology medicine.anatomical_structure chemistry 030220 oncology & carcinogenesis Mutation Chediak-Higashi Syndrome Lysosomes Histamine |
| Zdroj: | International Immunopharmacology. 69:202-212 |
| ISSN: | 1567-5769 |
| Popis: | Chèdiak-Higashi syndrome is a rare autosomal recessive disease that causes hypopigmentation, recurrent infections, mild coagulation defects and neurological problems. Beige mice carry a mutation in the lysosome trafficking regulator (LYST) gene and display some of the key characteristics of human Chèdiak-Higashi syndrome, in particular, a high susceptibility to infection due to aberrant natural killer (NK) cell and polymorphonuclear leucocyte function. Morphological analysis of beige mice reveals the presence of enlarged lysosomes in a variety of cell types, including leucocytes, hepatocytes, fibroblasts and renal tubule cells. To examine the process of granule maturation and degranulation in beige mice mast cells, morphological studies have been conducted using a combination of electrophysiological techniques; however, few functional studies have been conducted with mast cells, such as mediator release. The aim of the present study was to determine the morphological and functional characteristics of skin and peritoneal mast cells and bone marrow-derived mast cells of homozygous (bg/bg) and heterozygous (bg/+) beige mice and wild-type (+/+) mice. The histamine concentration was lower in the peritoneal and bone marrow-derived mast cells of bg/bg mice compared with those of bg/+ and +/+ mice, but the histamine release response was potentiated. In vivo studies of passive cutaneous anaphylaxis showed no differences between bg/bg mice and either bg/+ or +/+ mice. Although bg/bg mast cells with enlarged granules display specific exocytotic processes in vitro, the consequences of mast cell activation in beige mice were similar to those of wild-type mice in vivo. |
| Databáze: | OpenAIRE |
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