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The Function of KDEL Receptors as UPR Genes in Disease.

ABSTRACT: 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.

SUBMITTER: Wires ES

PROVIDER: S-EPMC8196686 | biostudies-literature | 2021 May

REPOSITORIES: biostudies-literature

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The Function of KDEL Receptors as UPR Genes in Disease.

Wires Emily S ES Trychta Kathleen A KA Kennedy Lacey M LM Harvey Brandon K BK

International journal of molecular sciences 20210521 11

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 ...[more]

PMID: 34063979

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