Biogenesis of lysosome-related organelles complex-1 subunit 1 pre-messenger RNA (BLOC1S1 pre-mRNA) (BLOC1S1 pre-mRNA)

Biogenesis of lysosome-related organelles complex-1 subunit 1 (BLOC1S1) pre-messenger RNA is a pivotal component of the cellular unfolded protein response (UPR). It is a primary substrate for Regulated IRE1-dependent decay (RIDD), a process where the ER stress sensor IRE1 alpha cleaves specific mRNAs to maintain proteostasis (PubMed: 21602803). The BLOC1S1 protein is involved in lysosome biogenesis and endosomal trafficking, and its RIDD-mediated degradation is a critical switch during prolonged ER stress (UniProt: Q9Y263). Dysregulation of this pathway is associated with metabolic diseases, including non-alcoholic fatty liver disease, and various malignancies where ER stress adaptation is crucial for tumor survival (PubMed: 25214444). Therapeutic targeting of BLOC1S1 pre-mRNA typically involves small molecule inhibitors of IRE1 alpha or experimental antisense oligonucleotides designed to modulate its stability. Understanding the BLOC1S1-RIDD axis provides insights into how cells balance organelle biogenesis with stress-induced mRNA decay.

Other names

GCN5L1BLOS1RT14Biogenesis of lysosomal organelles complex-1 subunit 1

Inhibition of Regulated IRE1-dependent decay (RIDD)