KDELR2
Description
The KDELR2 (KDEL endoplasmic reticulum protein retention receptor 2) is a protein-coding gene located on chromosome 7.
KDELR2 is a human gene that encodes ER lumen protein retaining receptor 2. This receptor plays a crucial role in retaining soluble proteins within the endoplasmic reticulum (ER). It achieves this by continuously retrieving these proteins from the cis-Golgi or a pre-Golgi compartment. Sorting of these proteins relies on a C-terminal tetrapeptide signal, lys-asp-glu-leu (KDEL) in animal cells. KDELR2 recognizes and binds proteins containing this signal, facilitating their return to the ER. KDELR2 is part of a family of genes homologous to ERD2 in yeast, which encodes a single sorting receptor. KDELR2 is closely related to KDELR1, sharing 83% identity in its protein product. Pathogenic variants in KDELR2 have been linked to Osteogenesis imperfecta.
KDELR2 is a membrane receptor that binds the K-D-E-L sequence motif in the C-terminal part of endoplasmic reticulum resident proteins, ensuring their retention within that compartment. It accomplishes this by participating in vesicle-mediated recycling of these proteins back from the Golgi apparatus. Binding is pH-dependent, with optimal activity occurring at pH 5-5.4. (UniProtKB:Q5ZKX9, PubMed:1325562, PubMed:18086916, PubMed:33053334)
KDELR2 is also known as ELP-1, ELP1, ERD2.2, OI21.
