PMID:26431026

Citation	Stadel, D, Millarte, V, Tillmann, KD, Huber, J, Tamin-Yecheskel, BC, Akutsu, M, Demishtein, A, Ben-Zeev, B, Anikster, Y, Perez, F, Dötsch, V, Elazar, Z, Rogov, V, Farhan, H and Behrends, C (2015) TECPR2 Cooperates with LC3C to Regulate COPII-Dependent ER Export. Mol. Cell 60:89-104
Abstract	Hereditary spastic paraplegias (HSPs) are a diverse group of neurodegenerative diseases that are characterized by axonopathy of the corticospinal motor neurons. A mutation in the gene encoding for Tectonin β-propeller containing protein 2 (TECPR2) causes HSP that is complicated by neurological symptoms. While TECPR2 is a human ATG8 binding protein and positive regulator of autophagy, the exact function of TECPR2 is unknown. Here, we show that TECPR2 associates with several trafficking components, among them the COPII coat protein SEC24D. TECPR2 is required for stabilization of SEC24D protein levels, maintenance of functional ER exit sites (ERES), and efficient ER export in a manner dependent on binding to lipidated LC3C. TECPR2-deficient HSP patient cells display alterations in SEC24D abundance and ER export efficiency. Additionally, TECPR2 and LC3C are required for autophagosome formation, possibly through maintaining functional ERES. Collectively, these results reveal that TECPR2 functions as molecular scaffold linking early secretion pathway and autophagy.
Links	PubMed Online version:10.1016/j.molcel.2015.09.010
Keywords	Adaptor Proteins, Signal Transducing/metabolism; Autophagy; Carrier Proteins/genetics; Carrier Proteins/metabolism; Endoplasmic Reticulum/metabolism; HeLa Cells; Humans; Microtubule-Associated Proteins/metabolism; Mutation; Nerve Tissue Proteins/genetics; Nerve Tissue Proteins/metabolism; Protein Transport; Spastic Paraplegia, Hereditary/genetics; Spastic Paraplegia, Hereditary/metabolism; Vesicular Transport Proteins/metabolism
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Annotations

Gene product	Qualifier	GO Term	Evidence Code	with/from	Aspect	Extension	Notes	Status
HUMAN:MLP3C		GO:0032527: protein exit from endoplasmic reticulum	ECO:0000315:		P		Immunofluorescence was employed to target the export of MAN2A1-SFB-GFP from the ER to the Golgi upon knockdown of MAP1LC3C. Results as shown in Fig 6E show significant reduction of ER export of MAN2A1-SFB-GFP.	complete CACAO 13066
HUMAN:TCPR2		GO:0032527: protein exit from endoplasmic reticulum	ECO:0000315:		P		TECPR2 is required for protein exit from ER.(Fig 6E) Organism: Human	complete CACAO 13076

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