Lysosome-related organelles

FASEB J. 2000 Jul;14(10):1265-78. doi: 10.1096/fj.14.10.1265.


Lysosomes 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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Albinism, Oculocutaneous / genetics
  • Animals
  • Antigen-Presenting Cells / physiology
  • Antigen-Presenting Cells / ultrastructure
  • Basophils / physiology
  • Basophils / ultrastructure
  • Blood Platelets / physiology
  • Blood Platelets / ultrastructure
  • Chediak-Higashi Syndrome / genetics
  • Cytoplasmic Granules / physiology
  • Drosophila / genetics
  • Humans
  • Lysosomes / genetics
  • Lysosomes / physiology*
  • Lysosomes / ultrastructure
  • Melanosomes / physiology
  • Membrane Fusion
  • Membrane Proteins / metabolism
  • Mice
  • Models, Biological
  • Neutrophils / physiology
  • Neutrophils / ultrastructure
  • Organelles / genetics
  • Organelles / physiology*
  • Organelles / ultrastructure


  • Membrane Proteins