The DOI matches its Crossref record; 61 of the 61 references it cites were found in a registry or an index.
Proprotein convertase cleavage liberates a fibrillogenic fragment of a resident glycoprotein to initiate melanosome biogenesis
Open accessAbstract
Lysosome-related organelles are cell type–specific intracellular compartments with distinct morphologies and functions. The molecular mechanisms governing the formation of their unique structural features are not known. Melanosomes and their precursors are lysosome-related organelles that are characterized morphologically by intralumenal fibrous striations upon which melanins are polymerized. The integral membrane protein Pmel17 is a component of the fibrils and can nucleate their formation in the absence of other pigment cell–specific proteins. Here, we show that formation of intralumenal fibrils requires cleavage of Pmel17 by a furin-like proprotein convertase (PC). As in the generation of amyloid, proper cleavage of Pmel17 liberates a lumenal domain fragment that becomes incorporated into the fibrils; longer Pmel17 fragments generated in the absence of PC activity are unable to form organized fibrils. Our results demonstrate that PC-dependent cleavage regulates melanosome biogenesis by controlling the fibrillogenic activity of a resident protein. Like the pathologic process of amyloidogenesis, the formation of other tissue-specific organelle structures may be similarly dependent on proteolytic activation of physiological fibrillogenic substrates.
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Self-citationBiogenesis and Function of Multivesicular Bodies
Piper, R. C., & Katzmann, D. J. (2007) · Annual Review of Cell and Developmental Biology · doi:10.1146/annurev.cellbio.23.090506.123319 · Cited by 771
Prions as adaptive conduits of memory and inheritance
Shorter, J., & Lindquist, S. (2005) · Nature Reviews Genetics · doi:10.1038/nrg1616 · Cited by 555
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Metadata
- Authors
- Berson, J. F., Theos, A. C., Harper, D. C., Tenza, D., Raposo, G., & Marks, M. S.
- Venue
- The Journal of Cell Biology
- Publisher
- Rockefeller University Press
- Published
- 2003-05-05
- Volume
- 161
- Issue
- 3
- Pages
- 521-533
- ISSN
- 0021-9525 (ISSN-L)
- 1540-8140
- OpenAlex
- W2063423234
- Cited by
- 287
- References
- 61
- FWCI
- 5.25
- Type
- journal article
- Language
- en
- Open access
- green
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- Berson, J. F., Theos, A. C., Harper, D. C., Tenza, D., Raposo, G., & Marks, M. S. (2003). Proprotein convertase cleavage liberates a fibrillogenic fragment of a resident glycoprotein to initiate melanosome biogenesis. The Journal of Cell Biology. https://doi.org/10.1083/jcb.200302072
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