ER lumen protein-retaining receptor 2

Definition

Membrane receptor that binds the K-D-E-L sequence motif in the C-terminal part of endoplasmic reticulum resident proteins and maintains their localization in that compartment by participating to their vesicle-mediated recycling back from the Golgi. Binding is pH dependent, and is optimal at pH 5-5.4 (By similarity).

References:

[1]. M J Lewis, et al. Sequence of a second human KDEL receptor. J Mol Biol. 1992 Aug 20;226(4):913-6. [Content Brief]

[2]. Irina Raykhel, et al. A molecular specificity code for the three mammalian KDEL receptors. J Cell Biol. 2007 Dec 17;179(6):1193-204. [Content Brief]

[3]. Fleur S van Dijk, et al. Interaction between KDELR2 and HSP47 as a Key Determinant in Osteogenesis Imperfecta Caused by Bi-allelic Variants in KDELR2. Am J Hum Genet. 2020 Nov 5;107(5):989-999. [Content Brief]

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