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latent TGF-beta binding protein 1L; LTBP-1; TGF beta-1-binding protein 1; TGF-beta1-BP-1 (LTBP1)
Function:
- may be involved in the assembly, secretion & targeting of TGFB1 to sites at which it is stored &/or activated
- may play role in controlling & directing the activity of TGFB1
- may have a structural role in the extracellular matrix (ECM)
- contains hydroxylated Asn
- isoform short N-terminus is blocked
- iron & 2-oxoglutarate dependent 3-hydroxylation of Asp & Asn is (R) stereospecific within EGF domains
- two intrachain disulfide bonds from the TB3 domain are rearranged upon TGFB1 binding, & form interchain bonds with TGFB1 propeptide, anchoring it to the extracellular matrix
- the large latent complex of TGFB1 from platelets is composed of the TGFB1 molecule non-covalently associated with a disulfide-bonded complex of a dimer of the N-terminal propeptide of the TGFB1 precursor & LTBP1
- LTBP1 does not bind directly to active TGFB1
- binds to FBN1 & FBN2
- interacts with ADAMTSL2
Structure:
- associates covalently with small latent TGF-beta complex via domain TB 3
- belongs to the LTBP family
- contains 18 EGF-like domains
- contains 4 TB (TGF-beta binding) domains
Compartment: secreted
Alternative splicing:
- named isoforms=5; long (LTBP-1L), short (LTBP-1S)
Expression: isoform long is found in fibroblasts
General
latent TGF-beta binding protein (LTBP)
phosphoprotein
Properties
SIZE: MW = 173 kD
entity length = 1595 aa
COMPARTMENT: extracellular compartment
MOTIF: signal sequence {1-23}
N-glycosylation site {N495}
consensus repeat {549-1463} (3)
Ser phosphorylation site {S1597}
Tyr phosphorylation site {Y1600}
Thr phosphorylation site {T1601}
EGF domain {501-1692} (18)
MOTIF: cysteine residue {C505}
MODIFICATION: cysteine residue {C516}
cysteine residue {C511}
MODIFICATION: cysteine residue {C525}
cysteine residue {C516}
MODIFICATION: cysteine residue {C505}
cysteine residue {C525}
MODIFICATION: cysteine residue {C511}
cysteine residue {C527}
MODIFICATION: cysteine residue {C540}
cysteine residue {C540}
MODIFICATION: cysteine residue {C527}
Database Correlations
OMIM 150390
UniProt Q14766
PFAM correlations
Entrez Gene 4052
Kegg hsa:4052
References
UniProt :accession Q14457