7r3m

Structural highlights

Disease

TGO1_HUMAN The disease may be caused by variants affecting the gene represented in this entry.

Function

TGO1_HUMAN Plays a role in the transport of cargos that are too large to fit into COPII-coated vesicles and require specific mechanisms to be incorporated into membrane-bound carriers and exported from the endoplasmic reticulum. This protein is required for collagen VII (COL7A1) secretion by loading COL7A1 into transport carriers. It may participate in cargo loading of COL7A1 at endoplasmic reticulum exit sites by binding to COPII coat subunits Sec23/24 and guiding SH3-bound COL7A1 into a growing carrier. Does not play a role in global protein secretion and is apparently specific to COL7A1 cargo loading. However, it may participate in secretion of other proteins in cells that do not secrete COL7A1. It is also specifically required for the secretion of lipoproteins by participating in their export from the endoplasmic reticulum (PubMed:27138255, PubMed:19269366). Required for correct assembly of COPII coat components at endoplasmic reticulum exit sites (ERES) and for the localization of SEC16A and membrane-bound ER-resident complexes consisting of MIA2 and PREB/SEC12 to ERES (PubMed:28442536).^{[1] [2] [3]}

References

1. ↑ Saito K, Chen M, Bard F, Chen S, Zhou H, Woodley D, Polischuk R, Schekman R, Malhotra V. TANGO1 facilitates cargo loading at endoplasmic reticulum exit sites. Cell. 2009 Mar 6;136(5):891-902. PMID:19269366 doi:10.1016/j.cell.2008.12.025
2. ↑ Santos AJ, Nogueira C, Ortega-Bellido M, Malhotra V. TANGO1 and Mia2/cTAGE5 (TALI) cooperate to export bulky pre-chylomicrons/VLDLs from the endoplasmic reticulum. J Cell Biol. 2016 May 9;213(3):343-54. PMID:27138255 doi:10.1083/jcb.201603072
3. ↑ Maeda M, Katada T, Saito K. TANGO1 recruits Sec16 to coordinately organize ER exit sites for efficient secretion. J Cell Biol. 2017 Jun 5;216(6):1731-1743. PMID:28442536 doi:10.1083/jcb.201703084