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

More general terms

• latent TGF-beta binding protein (LTBP)
• phosphoprotein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=4052
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:4052
• OMIM: https://mirror.omim.org/entry/150390
• UniProt: http://www.uniprot.org/uniprot/Q14766.html