Latent transforming growth factor-beta 1 binding protein 1 (LTBP-1), a heavy glycoprotein, is part of the platelet-derived TGF-beta 1 complex. LTBP-1 serves as an anchor for latent TGF-beta in the extracellular matrix and is a component of microfibrillar structures. Cleavage of LTBP results in LTBP-1, which may sequester latent TGF-beta in the extracellular matrix and regulate its activation. LTBP-1 mRNA is enriched in ovarian carcinoma tissues and highly expressed in serous and mucinous adenocarcinomas.
Function:
May 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).
Subunit:
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 and LTBP1. LTBP1 does not bind directly to active TGFB1. Binds to FBN1 and FBN2. Interacts with ADAMTSL2.
Subcellular Location:
Secreted.
Tissue Specificity:
Isoform Long is found in fibroblasts.
Post-translational modifications:
Contains hydroxylated asparagine residues.
Isoform Short N-terminus is blocked.
The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.
Similarity:
Belongs to the LTBP family.
Contains 18 EGF-like domains.
Contains 4 TB (TGF-beta binding) domains.
SWISS:
Q14766
Gene ID:
4052
Database links:
Entrez Gene: 4052 Human
Entrez Gene: 268977 Mouse
Entrez Gene: 59107 Rat
Omim: 150390 Human
SwissProt: Q14766 Human
SwissProt: Q8CG19 Mouse
SwissProt: Q00918 Rat
Unigene: 619315 Human
Unigene: 269747 Mouse
Unigene: 40942 Rat
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