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- W2930463924 endingPage "435" @default.
- W2930463924 startingPage "404" @default.
- W2930463924 abstract "Lysosome-related organelles (LROs) comprise a diverse group of cell type-specific, membrane-bound subcellular organelles that derive at least in part from the endolysosomal system but that have unique contents, morphologies and functions to support specific physiological roles. They include: melanosomes that provide pigment to our eyes and skin; alpha and dense granules in platelets, and lytic granules in cytotoxic T cells and natural killer cells, which release effectors to regulate hemostasis and immunity; and distinct classes of lamellar bodies in lung epithelial cells and keratinocytes that support lung plasticity and skin lubrication. The formation, maturation and/or secretion of subsets of LROs are dysfunctional or entirely absent in a number of hereditary syndromic disorders, including in particular the Hermansky-Pudlak syndromes. This review provides a comprehensive overview of LROs in humans and model organisms and presents our current understanding of how the products of genes that are defective in heritable diseases impact their formation, motility and ultimate secretion." @default.
- W2930463924 created "2019-04-11" @default.
- W2930463924 creator A5008158531 @default.
- W2930463924 creator A5018983964 @default.
- W2930463924 creator A5038636176 @default.
- W2930463924 creator A5082450551 @default.
- W2930463924 date "2019-05-29" @default.
- W2930463924 modified "2023-10-17" @default.
- W2930463924 title "The road to lysosome‐related organelles: Insights from Hermansky‐Pudlak syndrome and other rare diseases" @default.
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