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TitleCryo-EM of wild-type and mutant PMEL amyloid cores reveals structural mechanism of pigment dispersion syndrome.
Journal, issue, pagesNat Commun, Vol. 16, Issue 1, Page 5411, Year 2025
Publish dateJul 1, 2025
AuthorsHaruaki Yanagisawa / Harumi Arai / Tony Wang / Hideyuki Miyazawa / Masahide Kikkawa / Toshiyuki Oda /
PubMed AbstractPMEL amyloids serve as essential scaffolds for melanin deposition in melanosomes, playing a crucial role in pigmentation. Despite their importance, the high-resolution structure of PMEL amyloids has ...PMEL amyloids serve as essential scaffolds for melanin deposition in melanosomes, playing a crucial role in pigmentation. Despite their importance, the high-resolution structure of PMEL amyloids has remained unresolved. Using cryo-electron microscopy, we determine near-atomic resolution structures of wild-type PMEL amyloid core, revealing two distinct polymorphic forms with structural features. We further investigate the pathogenic G175S mutation associated with pigment dispersion syndrome (PDS). Structural analysis reveales that G175S introduces an additional hydrogen bond, stabilizing an alternative fibril conformation. In vitro, the G175S mutant exhibits a fourfold increase in polymerization efficiency compared to the wild type. In cells, G175S expression resultes in a twofold increase in intracellular amyloid content and a ~70% increase in extracellular amyloids, without altering melanosome morphology or number. These results indicate that the G175S mutation enhances amyloidogenesis within melanosomes, elevating amyloid load and potentially contributing to PDS pathophysiology. This study provides molecular insights into PMEL amyloid formation, highlighting its structural diversity and dysregulation in pigmentation disorders.
External linksNat Commun / PubMed:40595665 / PubMed Central
MethodsEM (single particle)
Resolution1.79 - 1.94 Å
Structure data

61782

9jst

EMDB-61782, PDB-9jst:
Wild-type native PMEL amyloid - polymorph 1
Method: EM (single particle) / Resolution: 1.79 Å

61783

9jsu

EMDB-61783, PDB-9jsu:
Wild-type native PMEL amyloid - polymorph 2
Method: EM (single particle) / Resolution: 1.79 Å

61784

9jsv

EMDB-61784, PDB-9jsv:
G175S mutant native PMEL amyloid
Method: EM (single particle) / Resolution: 1.79 Å

61785

9jsw

EMDB-61785, PDB-9jsw:
Wild-type PMEL CAF amyloid -in vitro polymerized
Method: EM (single particle) / Resolution: 1.94 Å

61786

9jsx

EMDB-61786, PDB-9jsx:
G175S PMEL CAF amyloid - in vitro polymerized
Method: EM (single particle) / Resolution: 1.79 Å

Source
  • homo sapiens (human)
KeywordsSTRUCTURAL PROTEIN / melanosome / melanoma / pigment / melanin / amyloid / glaucoma

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