MGP Database

MGP001843

UniProt Annotations

Entry Information
Gene Namelatent transforming growth factor beta binding protein 1
Protein EntryLTBP1_HUMAN
UniProt IDQ14766
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=5; Name=Long; Synonyms=LTBP-1L; IsoId=Q14766-1; Sequence=Displayed; Name=Short; Synonyms=LTBP-1S; IsoId=Q14766-2, P22064-1; Sequence=VSP_036963, VSP_036964; Name=3; IsoId=Q14766-3; Sequence=VSP_036963, VSP_036964, VSP_036965; Note=No experimental confirmation available.; Name=4; IsoId=Q14766-4; Sequence=VSP_040125; Name=5; IsoId=Q14766-5; Sequence=VSP_036963, VSP_036964, VSP_040125; Note=No experimental confirmation available.;
DomainAssociates covalently with small latent TGF-beta complex via domain TB 3.
FunctionMay be involved in the assembly, secretion and targeting of TGFB1 to sites at which it is stored and/or activated. May play critical roles in controlling and directing the activity of TGFB1. May have a structural role in the extra cellular matrix (ECM).
PtmContains hydroxylated asparagine residues. {ECO:0000269|PubMed:2350783}.
PtmIsoform Short N-terminus is blocked.
PtmThe iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains. {ECO:0000250}.
PtmTwo intrachain disulfide bonds from the TB3 domain are rearranged upon TGFB1 binding, and form interchain bonds with TGFB1 propeptide, anchoring it to the extracellular matrix.
Sequence CautionSequence=BAD92038.1; Type=Erroneous initiation; Note=Translation N-terminally shortened.; Evidence={ECO:0000305};
SimilarityBelongs to the LTBP family. {ECO:0000305}.
SimilarityContains 18 EGF-like domains. {ECO:0000255|PROSITE- ProRule:PRU00076}.
SimilarityContains 4 TB (TGF-beta binding) domains. {ECO:0000305}.
Subcellular LocationSecreted.
SubunitThe 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 and LTBP1. LTBP1 does not bind directly to active TGFB1. Binds to FBN1 and FBN2. Interacts with ADAMTSL2. {ECO:0000269|PubMed:10930463, ECO:0000269|PubMed:12429738, ECO:0000269|PubMed:18677313}.
Tissue SpecificityIsoform Long is found in fibroblasts.
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