Protein transport protein Sec61 subunit alpha isoform 1 (SEC61A1)

SEC61A1 encodes the alpha subunit of the SEC61 complex, a heteromeric protein channel anchored in the ER membrane. The Sec61 channel is the principal route for cotranslational and some posttranslational transport of secretory and transmembrane proteins into the ER, where they fold and mature. SEC61A1 also forms the ribosome receptor and a gated pore in the ER membrane, critical for correct targeting and movement of nascent polypeptides. The protein has ten transmembrane domains that create a central channel capable of dynamic opening and closing for substrate passage. SEC61A1 also controls passive ER Ca²⁺ leakage, contributing to cellular calcium balance and linking it to stress pathways such as the unfolded protein response. Pathogenic mutations in SEC61A1 are associated with genetic kidney diseases and immunodeficiencies, and the target is being explored for anticancer therapy via specific channel inhibitors.

Other names

Protein transport protein Sec61 subunit alpha isoform 1SEC61A1Sec61 alpha-1SEC61ASec61 alpha 1 subunitADTKD5CVID15HNFJ4HSEC61SEC61

Allosteric inhibition or blockage of the Sec61 channel, preventing protein translocation into the ER; Modulating ER calcium leakage and UPR (unfolded protein response)