FRAS1-related extracellular matrix protein 2 (ECM3 homolog, FREM2)

Function: 1) extracellular matrix protein 2) maintenance of skin epithelium 3) maintenance of renal epithelia 4) role in epidermal adhesion 5) Calx-beta domains bind Ca⁺² inducing a conformational shift Structure: 1) belongs to the FRAS1 family 2) contains 5 Calx-beta domains - contains 12 CSPG repeats (NG2 repeats) Compartment: membrane Alternative splicing: named isoforms=2 Pathology: - defects in FREM2 are a cause of Fraser syndrome

General

Ca+2 binding protein matrix protein membrane protein

Properties

SIZE: MW = 351 kD entity length = 3169 aa COMPARTMENT: extracellular matrix MOTIF: signal sequence {1-46} CSPG {298-419} MOTIF: N-glycosylation site {N358} CSPG {420-541} CSPG {542-681} CSPG {682-811} CSPG {812-921} CSPG {922-1052} CSPG {1053-1171} CSPG {1172-1284} MOTIF: N-glycosylation site {N1244} CSPG {1285-1400} MOTIF: N-glycosylation site {N1369} CSPG {1401-1514} CSPG {1515-1633} MOTIF: N-glycosylation site {N1584} CSPG {1634-1758} MOTIF: N-glycosylation site {N1741} Calx-beta 1 {1759-1858} Calx-beta 2 {1871-1982} Calx-beta 3 {1997-2103} Calx-beta 4 {2118-2220} Calx-beta 5 {2238-2342} transmembrane domain {3114-3134}

Database Correlations

OMIM correlations MORBIDMAP 608945 UniProt Q5SZK8 Pfam PF03160