latent TGF-beta binding protein 3 (LTBP-3)

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 - contains hydroxylated Asn (putative) - 2 intrachain disulfide bonds from the TB3 domain are rearranged upon TGFB1 binding, & form interchain bonds with TGFB1 propeptide, anchoring it to the extracellular matrix - forms part of the large latent TGF beta precursor complex - removal is required for activation of the complex Structure: - belongs to the LTBP family - contains 13 EGF-like domains - contains 4 TB (TGF-beta binding) domains Compartment: - secreted (putative) - secretion occurs after coexpression with TGFB1 & requires complexing with Cys-33 of the TGFB1 propeptide (putative) Alternative splicing: named isoforms=2 Expression: - isoform 1 is strongly expressed in pancreas & liver - isoform 2 is expressed prominently in heart, skeletal muscle, prostate, testis, small intestine & ovary Pathology: - defects in LTBP3 are the cause of tooth agenesis selective type 6e

Properties

SIZE: MW = 195 kD entity length = 1821 aa COMPARTMENT: extracellular compartment MOTIF: signal sequence {1-42} glycine-rich region {3-166} MOTIF: N-glycosylation site {N88} EGF domain {108-140} consensus repeat {286-329} N-glycosylation site {N348} EGF domain {354-394} (5) MOTIF: cysteine residue {C358} MODIFICATION: cysteine residue {C369} cysteine residue {C364} MODIFICATION: cysteine residue {C378} cysteine residue {C369} MODIFICATION: cysteine residue {C358} cysteine residue {C378} MODIFICATION: cysteine residue {C364} cysteine residue {C380} MODIFICATION: cysteine residue {C393} cysteine residue {C393} MODIFICATION: cysteine residue {C380} cysteine-rich region {577-893} MOTIF: EGF domain {615-658} (5) MOTIF: cysteine residue {C619} MODIFICATION: cysteine residue {C631} cysteine residue {C624} MODIFICATION: cysteine residue {C640} cysteine residue {C631} MODIFICATION: cysteine residue {C619} cysteine residue {C640} MODIFICATION: cysteine residue {C624} cysteine residue {C642} MODIFICATION: cysteine residue {C657} cysteine residue {C657} MODIFICATION: cysteine residue {C642} EGF domain {825-864} (1) MOTIF: cysteine residue {C829} MODIFICATION: cysteine residue {C840} cysteine residue {C835} MODIFICATION: cysteine residue {C849} cysteine residue {C840} MODIFICATION: cysteine residue {C829} N-glycosylation site {N844} cysteine residue {C849} MODIFICATION: cysteine residue {C835} cysteine residue {C851} MODIFICATION: cysteine residue {C863} cysteine residue {C863} MODIFICATION: cysteine residue {C851}

Database Correlations

OMIM correlations MORBIDMAP 602090 UniProt Q9NS15 PFAM correlations Entrez Gene 4054 Kegg hsa:4054

References

UniProt :accession Q9NS15