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secreted frizzled-related protein 3; sFRP-3; Frezzled; Fritz; FrzB-1 (FRZB FIZ FRE FRP FRZB1 SFRP3)
Function:
- soluble frizzled-related proteins (sFRPS) function as modulators of Wnt signaling through direct interaction with Wnts
- they have a role in regulating cell growth & differentiation in specific cell types
- SFRP3/FRZB appears to be involved in limb skeletogenesis
- antagonist of Wnt8 signaling
- regulates chondrocyte maturation & long bone development
Structure:
- the FZ domain is involved in binding with Wnt ligands
- belongs to the secreted frizzled-related protein (sFRP) family
- contains 1 FZ (frizzled) domain
- contains 1 NTR domain
Compartment: secreted (probable)
Expression:
- expressed primarily in the cartilagenous cores of the long bone during embryonic & fetal development & in the appendicular skeleton (6-13 weeks)
- at 13 weeks of gestation, transcripts were present in early chondroblasts of the tarsal bones of the foot, the carpal bones of the hands & the epiphysis of long bones
- highly expressed in placenta & heart > brain, skeletal muscle, kidney & pancreas > lung & liver
Pathology:
- defects in FRZB are associated with susceptibility to osteoarthritis type 1
Notes:
- first purified as chondrogenic factor found in cartilage
General
secreted frizzled-related protein (sFRP) family
Properties
SIZE: entity length = 325 aa
MW = 36 kD
COMPARTMENT: extracellular compartment
MOTIF: signal sequence {1-32}
frizzled domain {33-150}
MOTIF: cysteine residue {C35}
MODIFICATION: cysteine residue {C96}
cysteine residue {C43}
MODIFICATION: cysteine residue {C89}
N-glycosylation site {N49}
cysteine residue {C80}
MODIFICATION: cysteine residue {C119}
cysteine residue {C89}
MODIFICATION: cysteine residue {C43}
cysteine residue {C96}
MODIFICATION: cysteine residue {C35}
cysteine residue {C108}
MODIFICATION: cysteine residue {C147}
cysteine residue {C112}
MODIFICATION: cysteine residue {C136}
cysteine residue {C119}
MODIFICATION: cysteine residue {C80}
cysteine residue {C136}
MODIFICATION: cysteine residue {C112}
cysteine residue {C147}
MODIFICATION: cysteine residue {C108}
NTR {178-298}
serine-rich region {301-318}
MOTIF: serine residue (SEVERAL)
Database Correlations
OMIM correlations
MORBIDMAP 605083
UniProt Q92765
PFAM correlations
Entrez Gene 2487
Kegg hsa:2487
References
- UniProt :accession Q92765
- Kawano Y & Kypta R
Secreted antagonists of the Wnt signalling pathway.
J Cell Sci 116:2627-2634, 2003
PMID: 12775774