Linking albinism and immunity: the secrets of secretory lysosomes

Science. 2004 Jul 2;305(5680):55-9. doi: 10.1126/science.1095291.

Abstract

Lysosomes are membrane-bound organelles that are found in all mammalian cells and contain hydrolases and lipases required for protein and membrane degradation. In many cells of the immune system, lysosomes also contain secretory proteins that can be released by regulated exocytosis in response to an external stimulus, providing different cell types with a wide range of effector functions. Melanosomes also use a lysosome-related organelle to secrete melanin for pigmentation. Links between albinism and immunity in patients have uncovered a number of key proteins required for lysosomal secretion and have revealed a versatile secretory mechanism that can be fine-tuned by distinct interactions in different cell types.

Publication types

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

MeSH terms

  • Albinism / genetics
  • Albinism / physiopathology*
  • Animals
  • Exocytosis
  • Humans
  • Immune System / metabolism*
  • Immunologic Deficiency Syndromes / genetics
  • Immunologic Deficiency Syndromes / immunology
  • Immunologic Deficiency Syndromes / physiopathology*
  • Lysosomes / metabolism*
  • Lysosomes / physiology
  • Melanins / metabolism
  • Melanocytes / metabolism
  • Melanosomes / metabolism*
  • Membrane Glycoproteins / physiology
  • Microtubule-Organizing Center / physiology
  • Mutation
  • Neurons / metabolism
  • Perforin
  • Pore Forming Cytotoxic Proteins
  • Proteins / physiology
  • Syndrome
  • T-Lymphocytes, Cytotoxic / immunology
  • T-Lymphocytes, Cytotoxic / metabolism
  • rab GTP-Binding Proteins / physiology
  • rab27 GTP-Binding Proteins

Substances

  • Melanins
  • Membrane Glycoproteins
  • Pore Forming Cytotoxic Proteins
  • Proteins
  • rab27 GTP-Binding Proteins
  • Perforin
  • RAB27A protein, human
  • rab GTP-Binding Proteins