Abstract

Melanosomes are tissue-specific lysosome-related organelles of pigment cells in which melanins are synthesized and stored. Analyses of the trafficking and fate of melanosomal components are beginning to reveal how melanosomes are formed through novel pathways from early endosomal intermediates. These studies unveil generalized structural and functional modifications of the endosomal system in specialized cells, and provide unexpected insights into the biogenesis of multivesicular bodies and how compartmentalization regulates protein refolding. Moreover, genetic disorders that affect the biogenesis of melanosomes and other lysosome-related organelles have shed light onto the molecular machinery that controls specialized endosomal sorting events.

Author affiliations

1. Institut Curie, Centre de Recherche, Paris, F-75248 France. Email: graca.raposo@curie.fr
2. CNRS, UMR144, Paris, F-75248 France.
3. Department of Pathology and Laboratory Medicine, University of Pennsylvania, 513 Stellar-Chance Laboratories, 422 Curie Boulevard, Philadelphia, Pennsylvania 19104-6100, USA.
   Email: marksm@mail.med.upenn.edu

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