ArticleProceedings of the National Academy of Sciences of the United States of America2014
C-terminal propeptide is required for fibrillin-1 secretion and blocks premature assembly through linkage to domains cbEGF41-43.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 37 citations in OpenAlex.
- A cadherin-integrin-ECM code for presomitic mesoderm fluidity.Development (Cambridge, England) · 2025Article
- Kallikrein-8 mediates furin-independent Activin-A precursor processing to stimulate tumor growth in melanoma.Nature communications · 2025Article
- Asprosin: its function as a novel endocrine factor in metabolic-related diseases.Journal of endocrinological investigation · 2024Review
- The extracellular matrix glycoprotein fibrillin-1 in health and disease.Frontiers in cell and developmental biology · 2023Review
- The Effects of Asprosin on Exercise-Intervention in Metabolic Diseases.Frontiers in physiology · 2022Review
- Structural studies of elastic fibre and microfibrillar proteins.Matrix biology plus · 2021Article
- Proof-of-Concept: Antisense Oligonucleotide Mediated Skipping of Fibrillin-1 Exon 52.International journal of molecular sciences · 2021Article
- Assembly assay identifies a critical region of human fibrillin-1 required for 10-12 nm diameter microfibril biogenesis.PloS one · 2021Article
- Fibrillin-1 and fibrillin-1-derived asprosin in adipose tissue function and metabolic disorders.Journal of cell communication and signaling · 2020Review
- [Expression, purification and functional assessment of asprosin inclusion body].Nan fang yi ke da xue xue bao = Journal of Southern Medical University · 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
- Aspartate/asparagine-β-hydroxylase crystal structures reveal an unexpected epidermal growth factor-like domain substrate disulfide pattern.Nature communications · 2019Article
- Article
- Truncated C-terminus of fibrillin-1 induces Marfanoid-progeroid-lipodystrophy (MPL) syndrome in rabbit.Disease models & mechanisms · 2018Article
- Structural and compositional diversity of fibrillin microfibrils in human tissues.The Journal of biological chemistry · 2018Article
- Fell-Muir Lecture: Fibrillin microfibrils: structural tensometers of elastic tissues?International journal of experimental pathology · 2017Review
- Expression of FBN1 during adipogenesis: Relevance to the lipodystrophy phenotype in Marfan syndrome and related conditions.Molecular genetics and metabolism · 2016Article
- Structure of the Elastin-Contractile Units in the Thoracic Aorta and How Genes That Cause Thoracic Aortic Aneurysms and Dissections Disrupt This Structure.The Canadian journal of cardiology · 2016Review
- A microfibril assembly assay identifies different mechanisms of dominance underlying Marfan syndrome, stiff skin syndrome and acromelic dysplasias.Human molecular genetics · 2015Article
- Genotype-phenotype Correlations of Ocular Posterior Segment Abnormalities in Marfan Syndrome.Ophthalmology scienceArticle
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
Fibrillin microfibrils are 10-12 nm diameter, extracellular matrix assemblies that provide dynamic tissues of metazoan species with many of their biomechanical properties as well as sequestering growth factors and cytokines. Assembly of fibrillin monomers into microfibrils is thought to occur at the cell surface, with initial steps including proprotein processing, multimerization driven by the C terminus, and the head-to-tail alignment of adjacent molecules. At present the mechanisms that regulate microfibril assembly are still to be elucidated. We have used structure-informed protein engineering to create a recombinant, GFP-tagged version of fibrillin-1 (GFP-Fbn) to study this process. Using HEK293T cells transiently transfected with GFP-Fbn constructs, we show that (i) the C-terminal propeptide is an essential requirement for the secretion of full-length fibrillin-1 from cells; (ii) failure to cleave off the C-terminal propeptide blocks the assembly of fibrillin-1 into microfibrils produced by dermal fibroblasts; and (iii) the requirement of the propeptide for secretion is linked to the presence of domains cbEGF41-43, because either deletion or exchange of domains in this region leads to cellular retention. Collectively, these data suggest a mechanism in which the propeptide blocks a key site at the C terminus to prevent premature microfibril assembly.
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