HDEL Antibody,StressMarq Biosciences Inc.,SMC-175D

The HDEL sequence (His-Asp-Glu-Leu) is a C-terminal ER retention signal found in many soluble endoplasmic reticulum (ER) resident proteins. It plays a critical role in maintaining ER homeostasis by ensuring that chaperones and folding enzymes are retained or retrieved from the Golgi apparatus back to the ER. In neurodegenerative diseases, where ER stress and protein misfolding are prominent pathological features, the HDEL motif is essential for the proper localization and function of ER-resident proteins such as GRP78, GRP94, and protein disulfide isomerases. Disruption of HDEL-mediated retention can impair the unfolded protein response (UPR), exacerbate proteotoxic stress, and contribute to neuronal dysfunction. Moreover, the efficiency of the HDEL retrieval system may decline with age or under chronic stress, potentially leading to the mislocalization of protective chaperones and increased vulnerability to neurodegeneration. Understanding the dynamics of HDEL-mediated trafficking offers insights into ER stress regulation and may inform therapeutic strategies aimed at enhancing proteostasis in neurodegenerative disorders.

H-D-E-L (his-asp-glu-leu), endoplasmic reticulum, luminal ER protein retention, KDELR1, Endoplasmic reticulum retention signal