Lysosomal trafficking regulator (LYST)

The lysosomal trafficking regulator (LYST) is a large cytosolic protein critical in regulating the trafficking and size of lysosomes and lysosome-related organelles (LROs)[1][2][3]. LYST is classified as a BEACH domain-containing protein (BDCP), a family of proteins involved in membrane trafficking and vesicular dynamics[2][3]. Structurally, LYST contains several domains, including ARM/HEAT repeats, a ConA-like lectin domain, a pleckstrin homology-like (PH-like) domain, a BEACH domain, and C-terminal WD40 repeats[2][3]. LYST influences lysosomal function by modulating vesicle fusion and fission events, maintaining lysosome homeostasis, and potentially serving as a scaffolding protein for SNARE complex and other signaling proteins involved in exocytosis[2][3]. Mutations in LYST disrupt these processes, leading to the formation of abnormally large lysosomes and organelles, defective exocytosis, and impaired immune cell function, resulting in the autosomal recessive disorder Chediak-Higashi syndrome[1][2][3]. There are presently no approved drugs specifically targeting LYST, nor is it recognized as a direct therapeutic target; its role is primarily genetic and mechanistic rather than pharmacologic[1][2][3].

Other names

Chediak-Higashi syndrome 1CHS1MauveBeige homologCHSlysosomal-trafficking regulatorLyst-1