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Lysosome-related organelles Dell'Angelica EC; Mullins C; Caplan S; Bonifacino JSFASEB J 2000[Jul]; 14 (10): 1265-78Lysosomes are membrane-bound cytoplasmic organelles involved in intracellular protein degradation. They contain an assortment of soluble acid-dependent hydrolases and a set of highly glycosylated integral membrane proteins. Most of the properties of lysosomes are shared with a group of cell type-specific compartments referred to as 'lysosome-related organelles', which include melanosomes, lytic granules, MHC class II compartments, platelet-dense granules, basophil granules, azurophil granules, and Drosophila pigment granules. In addition to lysosomal proteins, these organelles contain cell type-specific components that are responsible for their specialized functions. Abnormalities in both lysosomes and lysosome-related organelles have been observed in human genetic diseases such as the Chediak-Higashi and Hermansky-Pudlak syndromes, further demonstrating the close relationship between these organelles. Identification of genes mutated in these human diseases, as well as in mouse and Drosophila: pigmentation mutants, is beginning to shed light on the molecular machinery involved in the biogenesis of lysosomes and lysosome-related organelles.|Albinism, Oculocutaneous/genetics[MESH]|Animals[MESH]|Antigen-Presenting Cells/physiology/ultrastructure[MESH]|Basophils/physiology/ultrastructure[MESH]|Blood Platelets/physiology/ultrastructure[MESH]|Chediak-Higashi Syndrome/genetics[MESH]|Cytoplasmic Granules/physiology[MESH]|Drosophila/genetics[MESH]|Humans[MESH]|Lysosomes/genetics/*physiology/ultrastructure[MESH]|Melanosomes/physiology[MESH]|Membrane Fusion[MESH]|Membrane Proteins/metabolism[MESH]|Mice[MESH]|Models, Biological[MESH]|Neutrophils/physiology/ultrastructure[MESH]|Organelles/genetics/*physiology/ultrastructure[MESH] |
