KDEL endoplasmic reticulum protein retention receptor 2 (KDELR2)

KDEL endoplasmic reticulum protein retention receptor 2 (KDELR2) is a seven-transmembrane domain receptor localized mainly in the Golgi and endoplasmic reticulum membranes. It recognizes and binds the KDEL tetrapeptide (Lys-Asp-Glu-Leu) sequence present at the C-terminus of resident ER proteins, facilitating their retrieval from the Golgi back to the ER and thus ensuring proper ER localization and proteostasis[1][2][3][4][5][7][8]. KDELR2 is part of a small family homologous to yeast ERD2, sharing high similarity with KDELR1. It also participates in cell signaling, activating various kinases and contributing to pathways such as the unfolded protein response, autophagy, and possibly modulation of cell proliferation and extracellular matrix degradation[2][5][6][9]. Mutations in KDELR2 can cause genetic disorders such as osteogenesis imperfecta, and aberrant expression or function has been linked to cancer progression and neurodegenerative diseases[1][4][6][7][9]. No direct pharmacological modulators of KDELR2 are approved or in use.

Other names

ER lumen protein-retaining receptor 2ERD2.2ELP-1KDEL receptor 2ELP1OI21ERD2-like protein 1(Lys-Asp-Glu-Leu) endoplasmic reticulum protein retention receptor 2ERD-2-like proteinKDEL (Lys-Asp-Glu-Leu) endoplasmic reticulum protein retention receptor 2

No established direct drug mechanisms; KDELR2 has been studied for its shuttling activity to improve drug delivery and antigen presentation, but no direct modulatory compounds are in use