RETR1 antibody binds to FAM134B (family with sequence similarity 134 member B), a transmembrane protein encoded by the RETREG1 gene. FAM134B is anchored in the ER membrane and facilitates ER-phagy by promoting the removal of damaged ER components via autophagosomes .
Alternative Names: RETREG1, FAM134B.
Cellular Role: Maintains ER integrity, supports collagen quality control, and ensures neuronal survival .
Pathological Relevance: Linked to hereditary sensory and autonomic neuropathy type II (HSAN-II) and SARS-CoV-2 infection .
Domains: N-terminal reticulon homology domain (RHD), LIR motif (LC3-interacting region), and a C-terminal cytoplasmic domain .
Mechanism: Bends ER membranes to initiate scission, recruits ATG8-family proteins (e.g., LC3) via its LIR motif, and directs ER fragments to autophagosomes .
ER-Phagy: Clears damaged ER under basal conditions to prevent proteotoxic stress.
Neuronal Survival: Essential for long-term survival of nociceptive and autonomic ganglion neurons .
Viral Interaction: SARS-CoV-2 ORF3A protein enhances RETREG1-mediated ER-phagy, exacerbating ER stress and inflammation .
Deficiency Impact: Loss of FAM134B causes ER expansion, impaired autophagy, and neurodegeneration, contributing to HSAN-II .
SARS-CoV-2: ORF3A protein hijacks RETREG1 to induce ER stress, facilitating viral replication and inflammatory responses .
ER-Phagy Regulation: Dysregulation of FAM134B may influence tumor progression by altering ER stress responses, though direct oncogenic roles are under investigation .
RETR1 antibodies (e.g., ab316161) are critical tools for:
Western Blotting: Detects ~64–66 kDa FAM134B in human cell lines (e.g., Jurkat, MOLT-4) .
Immunocytochemistry (ICC): Localizes FAM134B to nuclei in HeLa cells .
Functional Studies: Evaluates ER-phagy dynamics and viral ER stress mechanisms .
While RETR1 antibodies are primarily research tools, modulating FAM134B activity holds therapeutic potential for: