Endoplasmic reticulum export, subcellular distribution, and fibril formation by Pmel17 require an intact N-terminal domain junction

Leonhardt, Ralf M.;Vigneron, Nathalie;Rahner, Christoph;Van den Eynde, Benoit;Cresswell, Peter
(2010) Journal of Biological Chemistry — Vol. 285, n° 21, p. 16166-16183 (2010)

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Authors
  • Leonhardt, Ralf M.Howard Hughes Medical Institute & Department of Immunobiology, Yale University School of Medicine, New Haven, CT, USA
    Author
  • Vigneron, NathalieUCLouvain
    Author
  • Rahner, ChristophHoward Hughes Medical Institute & Department of Cell Biology, Yale University School of Medicine, New Haven, CT, USA
    Author
  • Van den Eynde, Benoitorcid-logoUCLouvain
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  • Cresswell, PeterHoward Hughes Medical Institute and Departments of Immunobiology & Cell Biology, Yale University School of Medicine, New Haven, CT, USA
    Author
Abstract
Pmel17 is a melanocyte/melanoma-specific protein that sub-cellularly localizes to melanosomes, where it forms a fibrillar matrix that serves for the sequestration of potentially toxic reaction intermediates of melanin synthesis and deposition of the pigment. As a key factor in melanosomal biogenesis, understanding intracellular trafficking and processing of Pmel17 is of central importance to comprehend how these organelles are formed, how they mature, and how they function in the cell. Using a series of deletion and missense mutants of Pmel17, we are able to show that the integrity of the junction between the N-terminal region and the polycystic kidney disease-like domain is highly crucial for endoplasmic reticulum export, subcellular targeting, and fibril formation by Pmel17 and thus for establishing functional melanosomes.
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Leonhardt, R. M., Vigneron, N., Rahner, C., Van den Eynde, B., & Cresswell, P. (2010). Endoplasmic reticulum export, subcellular distribution, and fibril formation by Pmel17 require an intact N-terminal domain junction. Journal of Biological Chemistry, 285(21), 16166-16183. https://doi.org/10.1074/jbc.M109.097725 (Original work published 2010)