CLN5 : CLN5 intracellular trafficking protein
Description
The CLN5 (CLN5 intracellular trafficking protein) is a protein-coding gene located on chromosome 13.
The CLN5 gene provides instructions for producing a protein whose function is not entirely understood. Cells produce an inactive CLN5 protein containing extra segments. This inactive form is called a preprotein. To become active, the extra segments must be removed, followed by additional processing steps. The active CLN5 protein is then transported to lysosomes, cell compartments responsible for digesting and recycling molecules. Research suggests that the CLN5 protein might play a role in how lysosomes break down or recycle damaged or unneeded proteins within cells.
The secreted form of CLN5 protein is an enzyme that catalyzes the synthesis of bis(monoacylglycero)phosphate (BMP) from two molecules of lysophosphatidylglycerol (LPG). BMP is also known as lysobisphosphatidic acid and plays a crucial role in the formation of intraluminal vesicles and in maintaining intracellular cholesterol balance. CLN5 protein exhibits specificity for LPG as the acyl donor and displays BMP synthase activity towards different forms of LPG, favoring longer chain lengths. It influences the retrograde trafficking of lysosomal sorting receptors, SORT1 and IGF2R, from endosomes to the trans-Golgi network by controlling the recruitment of the retromer complex to the endosomal membrane. CLN5 protein regulates the localization and activation of RAB7A, essential for recruiting the retromer complex to the endosomal membrane.
CLN5 is also known as -.
