ArticleThe EMBO journal1997
Solution structure of the transforming growth factor beta-binding protein-like module, a domain associated with matrix fibrils.
Article in The EMBO journal, 1997. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 46 papers.
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Who cites it
46 citing papers in PubMed, 121 citations in OpenAlex.
- Genotype-associated structural and nanomechanical alterations of aortic fibrillin-1 microfibrils in Marfan syndrome.Matrix biology plus · 2026Article
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- Characterisation of Type-1 Fibrillinopathies in a Sri Lankan Cohort: Genotype-Phenotype Correlations and Novel FBN1 Variants.Molecular syndromology · 2025Article
- A Novel Missense Mutation in the TGF-β-binding Protein-Like Domain 3 ofAdvanced biomedical research · 2023Article
- Cardiac Complications in Marfan Syndrome: A Review.Cureus · 2022Review
- The fibrillinopathies: New insights with focus on the paradigm of opposing phenotypes for both FBN1 and FBN2.Human mutation · 2022Review
- Structural studies of elastic fibre and microfibrillar proteins.Matrix biology plus · 2021Article
- LTBP4 in Health and Disease.Genes · 2021Review
- Assembly assay identifies a critical region of human fibrillin-1 required for 10-12 nm diameter microfibril biogenesis.PloS one · 2021Article
- Autosomal Recessive Cutis Laxa 1C Mutations Disrupt the Structure and Interactions of Latent TGFβ Binding Protein-4.Frontiers in genetics · 2021Article
- Connective Tissue Disorders and Cardiovascular Complications: The Indomitable Role of Transforming Growth Factor-β Signaling.Advances in experimental medicine and biology · 2021Article
- Pathophysiology and Pathogenesis of Marfan Syndrome.Advances in experimental medicine and biology · 2021Review
- A Novel Splicing Mutation in theFrontiers in genetics · 2020Article
- The role of fibrillin and microfibril binding proteins in elastin and elastic fibre assembly.Matrix biology : journal of the International Society for Matrix Biology · 2019Review
- Insights Into Enhanced Complement Activation by Structures of Properdin and Its Complex With the C-Terminal Domain of C3b.Frontiers in immunology · 2019Article
- LTBPs in biology and medicine: LTBP diseases.Matrix biology : journal of the International Society for Matrix Biology · 2018Review
- Editorial Commentary: Understanding Marfan syndrome, or "how not to invent the light bulb".Trends in cardiovascular medicine · 2016Article
- Fibrillin-containing microfibrils are key signal relay stations for cell function.Journal of cell communication and signaling · 2015Article
- A microfibril assembly assay identifies different mechanisms of dominance underlying Marfan syndrome, stiff skin syndrome and acromelic dysplasias.Human molecular genetics · 2015Article
- ¹H, ¹³C and ¹⁵N assignments of the four N-terminal domains of human fibrillin-1.Biomolecular NMR assignments · 2014Article
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
Here we describe the high resolution nuclear magnetic resonance (NMR) structure of a transforming growth factor beta (TGF-beta)-binding protein-like (TB) domain, which comes from human fibrillin-1, the protein defective in the Marfan syndrome (MFS). This domain is found in fibrillins and latent TGF-beta-binding proteins (LTBPs) which are localized to fibrillar structures in the extracellular matrix. The TB domain manifests a novel fold which is globular and comprises six antiparallel beta-strands and two alpha-helices. An unusual cysteine triplet conserved in the sequences of TB domains is localized to the hydrophobic core, at the C-terminus of an alpha-helix. The structure is stabilized by four disulfide bonds which pair in a 1-3, 2-6, 4-7, 5-8 pattern, two of which are solvent exposed. Analyses of MFS-causing mutations and the fibrillin-1 cell-binding RGD site provide the first clues to the surface specificity of TB domain interactions. Modelling of a homologous TB domain from LTBP-1 (residues 1018-1080) suggests that hydrophobic contacts may play a role in its interaction with the TGF-beta1 latency-associated peptide.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.