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The <jats:styled-content style=\"fixed-case\">PMEL<\/jats:styled-content> fibrils are required for optimal pigment cell function, as animals that either lack <jats:styled-content style=\"fixed-case\">PMEL<\/jats:styled-content> expression or express mutant <jats:styled-content style=\"fixed-case\">PMEL<\/jats:styled-content> variants show varying degrees of hypopigmentation and pigment cell inviability. The <jats:styled-content style=\"fixed-case\">PMEL<\/jats:styled-content> fibrils have biophysical properties of amyloid, a protein fold that is frequently associated with neurodegenerative and other diseases. However, <jats:styled-content style=\"fixed-case\">PMEL<\/jats:styled-content> is one of a growing number of non\u2010pathogenic amyloid proteins that contribute to the function of the cell and\/or organism that produces them. Understanding how <jats:styled-content style=\"fixed-case\">PMEL<\/jats:styled-content> generates amyloid in a non\u2010pathogenic manner might provide insights into how to avoid toxicity due to pathological amyloid formation. In this review, we summarize and reconcile data concerning the fate of <jats:styled-content style=\"fixed-case\">PMEL<\/jats:styled-content> from its site of synthesis in the endoplasmic reticulum to newly formed melanosomes and the role of distinct <jats:styled-content style=\"fixed-case\">PMEL<\/jats:styled-content> subdomains in trafficking and amyloid fibril formation. We then discuss how its progression through the secretory pathway into the endosomal system might allow for the regulated and non\u2010toxic conversion of <jats:styled-content style=\"fixed-case\">PMEL<\/jats:styled-content> into an ordered amyloid polymer.<\/jats:p>","DOI":"10.1111\/pcmr.12067","type":"journal-article","created":{"date-parts":[[2013,1,25]],"date-time":"2013-01-25T17:05:07Z","timestamp":1359133507000},"page":"300-315","source":"Crossref","is-referenced-by-count":191,"title":["<scp>PMEL<\/scp>: a pigment cell\u2010specific model for functional amyloid formation"],"prefix":"10.1111","volume":"26","author":[{"given":"Brenda","family":"Watt","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Department of Physiology, and Cell and Molecular Biology Graduate Group Perelman School of Medicine University of Pennsylvania Philadelphia PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"van 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