期刊
JOURNAL OF CELL BIOLOGY
卷 209, 期 4, 页码 563-577出版社
ROCKEFELLER UNIV PRESS
DOI: 10.1083/jcb.201410026
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资金
- National Institutes of Health grants from the National Eye Institute [R01 EY015625, P30 EY001583]
- National Institutes of Health grants from the National Institute of General Medical Sciences [R01 GM108807]
- Institut Curie
- Centre National de la Recherche Scientifique
- Wellcome Trust [078327]
- American Heart Association [0625437U]
- Wellcome Trust-Department of Biotechnology India Alliance Senior Fellowship [500122/Z/09/Z]
- Institutional Research and Academic Career Development Award postdoctoral fellowship [K12 GM081259]
- National Institutes of Health [F32 AR062476]
Hermansky-Pudlak syndrome (HPS) is a group of disorders characterized by the malformation of lysosome-related organelles, such as pigment cell melanosomes. Three of nine characterized HPS subtypes result from mutations in subunits of BLOC-2, a protein complex with no known molecular function. In this paper, we exploit melanocytes from mouse HPS models to place BLOC-2 within a cargo transport pathway from recycling endosomal domains to maturing melanosomes. In BLOC-2-deficient melanocytes, the melanosomal protein TYRP1 was largely depleted from pigment granules and underwent accelerated recycling from endosomes to the plasma membrane and to the Golgi. By live-cell imaging, recycling endosomal tubules of wild-type melanocytes made frequent and prolonged contacts with maturing melanosomes; in contrast, tubules from BLOC-2-deficient cells were shorter in length and made fewer, more transient contacts with melanosomes. These results support a model in which BLOC-2 functions to direct recycling endosomal tubular transport intermediates to maturing melanosomes and thereby promote cargo delivery and optimal pigmentation.
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