Universität zu Köln

Daverkausen-Fischer, Lea, Draga, Margarethe and Proels, Felicitas (2022). Regulation of Translation, Translocation, and Degradation of Proteins at the Membrane of the Endoplasmic Reticulum. Int. J. Mol. Sci., 23 (10). BASEL: MDPI. ISSN 1422-0067

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Abstract

The endoplasmic reticulum (ER) of mammalian cells is the central organelle for the maturation and folding of transmembrane proteins and for proteins destined to be secreted into the extracellular space. The proper folding of target proteins is achieved and supervised by a complex endogenous chaperone machinery. BiP, a member of the Hsp70 protein family, is the central chaperone in the ER. The chaperoning activity of BiP is assisted by ER-resident DnaJ (ERdj) proteins due to their ability to stimulate the low, intrinsic ATPase activity of BiP. Besides their co-chaperoning activity, ERdj proteins also regulate and tightly control the translation, translocation, and degradation of proteins. Disturbances in the luminal homeostasis result in the accumulation of unfolded proteins, thereby eliciting a stress response, the so-called unfolded protein response (UPR). Accumulated proteins are either deleterious due to the functional loss of the respective protein and/or due to their deposition as intra- or extracellular protein aggregates. A variety of metabolic diseases are known to date, which are associated with the dysfunction of components of the chaperone machinery. In this review, we will delineate the impact of ERdj proteins in controlling protein synthesis and translocation under physiological and under stress conditions. A second aspect of this review is dedicated to the role of ERdj proteins in the ER-associated degradation pathway, by which unfolded or misfolded proteins are discharged from the ER. We will refer to some of the most prominent diseases known to be based on the dysfunction of ERdj proteins.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Daverkausen-Fischer, Lea UNSPECIFIED UNSPECIFIED UNSPECIFIED Draga, Margarethe UNSPECIFIED UNSPECIFIED UNSPECIFIED Proels, Felicitas UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-658391
DOI:	10.3390/ijms23105576
Journal or Publication Title:	Int. J. Mol. Sci.
Volume:	23
Number:	10
Date:	2022
Publisher:	MDPI
Place of Publication:	BASEL
ISSN:	1422-0067
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language ER MEMBRANE; MECHANISTIC INSIGHTS; DISULFIDE REDUCTASE; MISFOLDED PROTEINS; SEC61 TRANSLOCON; RESIDENT PROTEIN; ATPASE ACTIVITY; DNAJ HOMOLOG; BIP; CHAPERONE Multiple languages Biochemistry & Molecular Biology; Chemistry, Multidisciplinary Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/65839