CNIH1 (cornichon family member 1) is a transmembrane cargo receptor that facilitates selective transport and maturation of proteins through the secretory pathway. Mechanistically, CNIH1 functions as a COPII-coated vesicle cargo receptor that associates with cargo proteins in the endoplasmic reticulum (ER) and promotes their transport via the secretory pathway 1. CNIH1 localizes primarily to the ER and interacts with TGF-alpha family proteins, regulating their processing and secretion; alterations in CNIH1 expression modulate TGF-alpha protein transport efficiency 2. CNIH1 also functions as an auxiliary subunit of AMPA-type glutamate receptors, though with lower potency than CNIH-2/3 variants in modulating receptor gating and trafficking 3. Disease relevance includes lung adenocarcinoma, where CNIH1 was identified as one of five hub vesicle-mediated transport genes in a prognostic risk model; elevated CNIH1 expression promoted LUAD cell growth and migration in vitro and correlated with immune checkpoint activation 4. A CNIH-2 genetic variant (rs4073582) was associated with gout susceptibility in genome-wide analysis, suggesting involvement in glutamate signaling regulation relevant to metabolic disease 5. Clinically, CNIH1 represents a potential therapeutic target for cancer immunotherapy combined with chemotherapy, and understanding its role in glutamate receptor trafficking may have implications for neurological conditions.