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FRAS1-related extracellular matrix protein 1 (protein QBRICK FREM1 C9orf154)
Function:
1) extracellular matrix protein
2) role in epidermal differentiation
3) required for epidermal adhesion during embryonic development
4) Calx-beta domain binds Ca+2 & undergos conformational shift
Structure:
1) belongs to the FRAS1 family
2) contains 1 C-type lectin domain
- contains 1 Calx-beta domain
- contains 12 CSPG (NG2) repeats
Compartment:
- secreted, extracellular matrix, basement membrane localizes at the basement membrane zone of embryonic epidermis & hair follicles
Alternative splicing: named isoforms=4
Note: Formerly called QBRICK because it contains 12 repeats: 'Q' stands for queen & is taken from the queen being the 12th in a suit of playing card, & 'BRICK' stands for the repeating unit
General
Ca+2 binding protein
glycoprotein
matrix protein
Properties
SIZE: MW = 244 kD
entity length = 2179 aa
COMPARTMENT: extracellular matrix
MOTIF: signal sequence {1-21}
Cell attachment site {199-201}
CSPG {275-394}
MOTIF: N-glycosylation site {N335}
CSPG {395-502}
CSPG {503-621}
MOTIF: N-glycosylation site {N560}
CSPG {622-758}
MOTIF: N-glycosylation site {N622}
CSPG {759-869}
CSPG {870-1005}
CSPG {1006-1129}
MOTIF: N-glycosylation site {N1014}
CSPG {1130-1256}
CSPG {1257-1375}
CSPG {1376-1487}
CSPG {1488-1608}
MOTIF: N-glycosylation site {N1566}
CSPG {1609-1729}
Calx-beta {1731-1830}
Cell attachment site {1907-1909}
C-type lectin {2060-2174}
MOTIF: cysteine residue {C2151}
MODIFICATION: cysteine residue {C2165}
cysteine residue {C2165}
MODIFICATION: cysteine residue {C2151}
Database Correlations
OMIM 608944
UniProt Q5H8C1
Pfam PF00059
References
UniProt :accession Q5H8C1