RETREG1

RETREG1 (reticulophagy regulator 1), commonly known as FAM134B, encodes a transmembrane protein localized to the endoplasmic reticulum (ER). It acts as a core mediator of reticulophagy—selective autophagic degradation of ER segments—via its LC3-interacting region (LIR), which binds autophagosomal LC3 proteins to drive ER turnover during stress or homeostasis maintenance.
Dysfunction of RETREG1 is linked to hereditary sensory and autonomic neuropathy type II (HSAN II), a disorder causing progressive peripheral sensory loss due to impaired ER quality control. Preclinical research explores modulators of its LC3-binding activity to correct reticulophagy defects, with potential applications for ER-related neurodegenerative and metabolic diseases, though no targeted drugs are yet approved.