The Function of KDEL Receptors as UPR Genes in Disease

The KDEL receptor retrieval pathway is essential for maintaining resident proteins in the endoplasmic reticulum (ER) lumen. ER resident proteins serve a variety of functions, including protein folding and maturation. Perturbations to the lumenal ER microenvironment, such as calcium depletion, can ca...

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Main Authors: Emily S. Wires, Kathleen A. Trychta, Lacey M. Kennedy, Brandon K. Harvey
Format: Article
Language:English
Published: MDPI AG 2021-05-01
Series:International Journal of Molecular Sciences
Subjects:
Online Access:https://www.mdpi.com/1422-0067/22/11/5436
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author Emily S. Wires
Kathleen A. Trychta
Lacey M. Kennedy
Brandon K. Harvey
author_facet Emily S. Wires
Kathleen A. Trychta
Lacey M. Kennedy
Brandon K. Harvey
author_sort Emily S. Wires
collection DOAJ
description The KDEL receptor retrieval pathway is essential for maintaining resident proteins in the endoplasmic reticulum (ER) lumen. ER resident proteins serve a variety of functions, including protein folding and maturation. Perturbations to the lumenal ER microenvironment, such as calcium depletion, can cause protein misfolding and activation of the unfolded protein response (UPR). Additionally, ER resident proteins are secreted from the cell by overwhelming the KDEL receptor retrieval pathway. Recent data show that KDEL receptors are also activated during the UPR through the IRE1/XBP1 signaling pathway as an adaptive response to cellular stress set forth to reduce the loss of ER resident proteins. This review will discuss the emerging connection between UPR activation and KDEL receptors as it pertains to ER proteostasis and disease states.
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spelling doaj.art-caa042c16c9e4db3b6d5dcfbe2929a1c2023-11-21T20:45:40ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672021-05-012211543610.3390/ijms22115436The Function of KDEL Receptors as UPR Genes in DiseaseEmily S. Wires0Kathleen A. Trychta1Lacey M. Kennedy2Brandon K. Harvey3Molecular Mechanisms of Cellular Stress and Inflammation, Intramural Research Program, National Institute on Drug Abuse, Baltimore, MD 21224, USAMolecular Mechanisms of Cellular Stress and Inflammation, Intramural Research Program, National Institute on Drug Abuse, Baltimore, MD 21224, USAMolecular Mechanisms of Cellular Stress and Inflammation, Intramural Research Program, National Institute on Drug Abuse, Baltimore, MD 21224, USAMolecular Mechanisms of Cellular Stress and Inflammation, Intramural Research Program, National Institute on Drug Abuse, Baltimore, MD 21224, USAThe KDEL receptor retrieval pathway is essential for maintaining resident proteins in the endoplasmic reticulum (ER) lumen. ER resident proteins serve a variety of functions, including protein folding and maturation. Perturbations to the lumenal ER microenvironment, such as calcium depletion, can cause protein misfolding and activation of the unfolded protein response (UPR). Additionally, ER resident proteins are secreted from the cell by overwhelming the KDEL receptor retrieval pathway. Recent data show that KDEL receptors are also activated during the UPR through the IRE1/XBP1 signaling pathway as an adaptive response to cellular stress set forth to reduce the loss of ER resident proteins. This review will discuss the emerging connection between UPR activation and KDEL receptors as it pertains to ER proteostasis and disease states.https://www.mdpi.com/1422-0067/22/11/5436KDEL receptorendoplasmic reticulumunfolded protein responseER resident proteinsdiseaseexodosis
spellingShingle Emily S. Wires
Kathleen A. Trychta
Lacey M. Kennedy
Brandon K. Harvey
The Function of KDEL Receptors as UPR Genes in Disease
International Journal of Molecular Sciences
KDEL receptor
endoplasmic reticulum
unfolded protein response
ER resident proteins
disease
exodosis
title The Function of KDEL Receptors as UPR Genes in Disease
title_full The Function of KDEL Receptors as UPR Genes in Disease
title_fullStr The Function of KDEL Receptors as UPR Genes in Disease
title_full_unstemmed The Function of KDEL Receptors as UPR Genes in Disease
title_short The Function of KDEL Receptors as UPR Genes in Disease
title_sort function of kdel receptors as upr genes in disease
topic KDEL receptor
endoplasmic reticulum
unfolded protein response
ER resident proteins
disease
exodosis
url https://www.mdpi.com/1422-0067/22/11/5436
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