FRAS1 Related Extracellular Matrix Protein 2 (FREM2)

FREM2 encodes a membrane protein that belongs to the FRAS1 family. This extracellular matrix protein is thought to be required for maintaining the integrity of the skin epithelium and the differentiated state of renal epithelia. The protein localizes to the basement membrane, forming a ternary complex that plays a role in epidermal-dermal interactions during morphogenetic processes. Mutations in this gene are associated with Fraser syndrome. The deduced proteins contain an N-terminal signal peptide, 12 chondroitin sulfate proteoglycan (CSPG) repeats, 5 calcium-binding loops similar to those of sodium-calcium exchangers, a transmembrane domain, and a C-terminal PDZ motif. They also have sites for N-glycosylation. FREM2 shares significant similarity with FRAS1 .