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latent TGF-beta binding protein 2; LTBP-2 (LTBP2, C14orf141, LTBP3)
Function:
- may play an integral structural role in elastic-fiber architectural organization &/or assembly
- contains hydroxylated Asn (putative)
- forms part of the large latent transforming growth factor beta precursor complex
- removal is essential for activation of the complex
- interacts with SDC4
Structure:
- associates covalently with small latent TGF-beta complex via repeat B & repeat C (putative)
- belongs to the LTBP family
- contains 20 EGF-like domains
- contains 4 TB (TGF-beta binding) domains
Compartment:
- secreted (putative)
- localized in nuchal ligament & aorta to the fibrillin- containing, microfibrillar component of elastic fibers (putative)
Expression:
- expressed in lung
- weakly expressed in heart, placenta, liver & skeletal muscle
Pathology:
- defects in LTBP2 are the cause of
a) primary congenital glaucoma type 3D
b) microspherophakia
General
latent TGF-beta binding protein (LTBP)
phosphoprotein
Properties
SIZE: entity length = 1821 aa
MW = 195 kD
COMPARTMENT: extracellular compartment
MOTIF: signal sequence {1-35}
binding site
SITE: 94-115
FOR-BINDING-OF: heparin
N-glycosylation site {N181}
EGF domain {187-219}
MOTIF: cysteine residue {C191}
MODIFICATION: cysteine residue {C201}
cysteine residue {C195}
MODIFICATION: cysteine residue {C207}
cysteine residue {C201}
MODIFICATION: cysteine residue {C191}
cysteine residue {C207}
MODIFICATION: cysteine residue {C195}
cysteine residue {C209}
MODIFICATION: cysteine residue {C218}
cysteine residue {C218}
MODIFICATION: cysteine residue {C209}
binding site
SITE: 232-249
FOR-BINDING-OF: heparin
N-glycosylation site {N343}
binding site
SITE: 344-354
FOR-BINDING-OF: heparin
Cell attachment site {375-377}
EGF domain {396-428}
MOTIF: cysteine residue {C400}
MODIFICATION: cysteine residue {C410}
cysteine residue {C404}
MODIFICATION: cysteine residue {C416}
cysteine residue {C410}
MODIFICATION: cysteine residue {C400}
cysteine residue {C416}
MODIFICATION: cysteine residue {C404}
cysteine residue {C418}
MODIFICATION: cysteine residue {C427}
N-glycosylation site {N421}
cysteine residue {C427}
MODIFICATION: cysteine residue {C418}
Ser phosphorylation site {S506}
TB 1 {552-604}
N-glycosylation site {N616}
EGF domain {622-662}
MOTIF: cysteine residue {C626}
MODIFICATION: cysteine residue {C637}
cysteine residue {C632}
MODIFICATION: cysteine residue {C646}
cysteine residue {C637}
MODIFICATION: cysteine residue {C626}
cysteine residue {C646}
MODIFICATION: cysteine residue {C632}
cysteine residue {C648}
MODIFICATION: cysteine residue {C661}
cysteine residue {C661}
MODIFICATION: cysteine residue {C648}
TB 2 {672-724}
N-glycosylation site {N811}
EGF domain {844-886}
MOTIF: cysteine residue {C848}
MODIFICATION: cysteine residue {C861}
cysteine-rich region {856-1372}
MOTIF: cysteine residue {C856}
MODIFICATION: cysteine residue {C870}
cysteine residue {C861}
MODIFICATION: cysteine residue {C848}
cysteine residue {C870}
MODIFICATION: cysteine residue {C856}
cysteine residue {C872}
MODIFICATION: cysteine residue {C885}
cysteine residue {C885}
MODIFICATION: cysteine residue {C872}
EGF domain {887-N1309} (11)
EGF domain {1345-1818} (4)
Database Correlations
OMIM correlations
MORBIDMAP 602091
UniProt Q14767
PFAM correlations
Entrez Gene 4053
Kegg hsa:4053
References
UniProt :accession Q14457