期刊
SCIENCE
卷 337, 期 6102, 页码 1668-1672出版社
AMER ASSOC ADVANCEMENT SCIENCE
DOI: 10.1126/science.1224947
关键词
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资金
- Telethon EM Core Facility [GTF08001]
- Telethon [GSP08002, GGP06166]
- AIRC [IG 8623]
- Programma Operativo Nazionale [01_00862]
- Telethon grant [GGP07075]
- NIH [NIH/AR053696]
- Plan Nacional [BFU2008-00414]
- Consolider [CSD2009-00016]
- Agencia de Gestio d'Ajuts Universitaris i de Recerca (AGAUR) Grups de Recerca Emergents
- AGAUR-Catalan Government [SGR2009-1488]
- European Research Council [268692]
- ICREA Funding Source: Custom
Newly synthesized proteins exit the endoplasmic reticulum (ER) via coat protein complex II (COPII) vesicles. Procollagen (PC), however, forms prefibrils that are too large to fit into typical COPII vesicles; PC thus needs large transport carriers, which we term megacarriers. TANGO1 assists PC packing, but its role in promoting the growth of megacarriers is not known. We found that TANGO1 recruited Sedlin, a TRAPP component that is defective in spondyloepiphyseal dysplasia tarda (SEDT), and that Sedlin was required for the ER export of PC. Sedlin bound and promoted efficient cycling of Sar1, a guanosine triphosphatase that can constrict membranes, and thus allowed nascent carriers to grow and incorporate PC prefibrils. This joint action of TANGO1 and Sedlin sustained the ER export of PC, and its derangement may explain the defective chondrogenesis underlying SEDT.
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