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

Function

• extracellular matrix protein
• maintenance of skin epithelium
• maintenance of renal epithelia
• role in epidermal adhesion
• Calx-beta domains bind Ca+2 inducing a conformational shift

Structure

• belongs to the FRAS1 family
• 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

More general terms

• matrix protein
• membrane protein
• Ca+2 binding protein

Database

• OMIM: https://mirror.omim.org/entry/219000
• OMIM: https://mirror.omim.org/entry/608945
• UniProt: http://www.uniprot.org/uniprot/Q5SZK8.html