Sedlin Controls the ER Export of Procollagen by Regulating the Sar1 Cycle

Overview

Journal Science

Specialty Science

Date 2012 Sep 29

PMID 23019651

Citations 91

Authors

Rossella Venditti

Tiziana Scanu

Michele Santoro

Giuseppe Di Tullio

Alexander Spaar

Renato Gaibisso

Galina V Beznoussenko

Alexander A Mironov

Alexander Mironov Jr

Leopoldo Zelante

Maria Rosaria Piemontese

Angelo Notarangelo

Vivek Malhotra

Barbara M Vertel

Cathal Wilson

Maria Antonietta De Matteis

Affiliations

Soon will be listed here.

Abstract

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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