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Review
. 2020 Mar;27(3):833-842.
doi: 10.1038/s41418-019-0444-0. Epub 2019 Oct 28.

ER-phagy and human diseases

Christian A Hübner  1 , Ivan Dikic  2   3   4
Affiliations
Review

ER-phagy and human diseases

Christian A Hübner et al. Cell Death Differ. 2020 Mar.

Abstract

Autophagy regulates the degradation of unnecessary or dysfunctional cellular components. This catabolic process requires the formation of a double-membrane vesicle, the autophagosome, that engulfs the cytosolic material and delivers it to the lysosome. Substrate specificity is achieved by autophagy receptors, which are characterized by the presence of at least one LC3-interaction region (LIR) or GABARAP-interaction motif (GIM). Only recently, several receptors that mediate the specific degradation of endoplasmic reticulum (ER) components via autophagy have been identified (the process known as ER-phagy or reticulophagy). Here, we give an update on the current knowledge about the role of ER-phagy receptors in health and disease.

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Conflict of interest statement

The authors declare that they have no conflict of interest.

Figures

Fig. 1
Fig. 1
Gross architecture of an eukaryotic cell and its ER. ERES endoplasmic reticulum exit sites, UPR unfolded protein response, ERAD endoplasmic reticulum associated protein degradation
Fig. 2
Fig. 2
The process of ER-phagy. ER-phagy receptors are recognized by LC3/GABARAP proteins, which allows the formation of the phagophore around the ER fragments. As the phagophore seals, it forms an autophagosome, which delivers the enclosed ER fragments to the lysosome for degradation
Fig. 3
Fig. 3
Structure and topology of known receptors for ER-phagy
Fig. 4
Fig. 4
Clinical presentation of a patient with the homozygous mutation p.S309X in FAM134B showing severe acro-osteolysis of fingers and toes and deformation of the skeleton of the foot because of recurrent painless bone fractures (HSAN2B)
See this image and copyright information in PMC

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