ArticleMatrix biology : journal of the International Society for Matrix Biology2011
Fibrillin-3 expression in human development.
Article in Matrix biology : journal of the International Society for Matrix Biology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers, 1 of them a synthesis that pooled it.
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Who cites it
39 citing papers in PubMed, 1 synthesis or guideline pooled it, 68 citations in OpenAlex.
- Evaluating reported candidate gene associations with polycystic ovary syndrome.Fertility and sterility · 2013Pooled it
- Copy number variation analysis in bicuspid aortic valve-related aortopathy identifies TBX20 as a contributing gene.European journal of human genetics : EJHG · 2019Trial
- The stromal microenvironment of ovary: a novel area in polycystic ovary syndrome.Journal of ovarian research · 2026Review
- Identification and evolutionary analysis of insect fibrillin with their closely related family proteins.PeerJ · 2026Article
- Fibrillin-1 G234D mutation in the hybrid1 domain causes tight skin associated with dysregulated elastogenesis and increased collagen cross-linking in mice.Matrix biology : journal of the International Society for Matrix Biology · 2025Article
- Purification of SlREC2 from wild-type tomato leaflets using immunoaffinity chromatography and immunoprecipitation.Frontiers in plant science · 2025Article
- Bridging Nature and Engineering: Protein-Derived Materials for Bio-Inspired Applications.Biomimetics (Basel, Switzerland) · 2024Review
- Genetic models of fibrillinopathies.Genetics · 2024Review
- The fibrillinopathies: New insights with focus on the paradigm of opposing phenotypes for both FBN1 and FBN2.Human mutation · 2022Review
- Article
- Further delineation of familial polycystic ovary syndrome (PCOS) via whole-exome sequencing: PCOS-related rare FBN3 and FN1 gene variants are identified.The journal of obstetrics and gynaecology research · 2022Article
- Case Report: A Novel Homozygous Missense Variant ofFrontiers in genetics · 2022Article
- Structural studies of elastic fibre and microfibrillar proteins.Matrix biology plus · 2021Article
- Altered Ocular Fibrillin Microfibril Composition in Mice With a Glaucoma-Causing Mutation of Adamts10.Investigative ophthalmology & visual science · 2021Article
- Fibrillin-1 and fibrillin-1-derived asprosin in adipose tissue function and metabolic disorders.Journal of cell communication and signaling · 2020Review
- Polygenic risk score for disability and insights into disability-related molecular mechanisms.GeroScience · 2019Article
- 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
- Limb- and tendon-specific Adamtsl2 deletion identifies a role for ADAMTSL2 in tendon growth in a mouse model for geleophysic dysplasia.Matrix biology : journal of the International Society for Matrix Biology · 2019Article
- Adamts10 inactivation in mice leads to persistence of ocular microfibrils subsequent to reduced fibrillin-2 cleavage.Matrix biology : journal of the International Society for Matrix Biology · 2019Article
- Elastic Fibers and Large Artery Mechanics in Animal Models of Development and Disease.Journal of biomechanical engineering · 2018Review
Corrections and comments
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Authors and funding
6 authors at 3 institutions in 3 countries.
Funding
Abstract
Fibrillin proteins are the major components of extracellular microfibrils found in many connective tissues. Fibrillin-1 and fibrillin-2 are well studied and mutations in these proteins cause a number of fibrillinopathies including Marfan syndrome and congenital contractural arachnodactyly, respectively. Fibrillin-3 was more recently discovered and is much less well characterized. Fibrillin-1 is expressed throughout life, whereas fibrillins-2 and -3 are thought to be primarily present during development. Here, we report detailed fibrillin-3 expression patterns in early human development. A polyclonal antiserum against a C-terminal recombinant half of human fibrillin-3 was produced in rabbit. Anti-fibrillin-3 antibodies were affinity-purified and antibodies cross-reacting with the other fibrillins were removed by absorption resulting in specific anti-fibrillin-3 antibodies. Immunohistochemical analyses with these purified antibodies demonstrate that fibrillin-3 is temporally expressed in numerous tissues relatively evenly from the 6th to the 12th gestational week. Fibrillin-3 was found spatially expressed in perichondrium, perineurium, perimysium, skin, developing bronchi, glomeruli, pancreas, kidney, heart and testis and at the prospective basement membranes in developing epithelia and endothelia. Double immunohistochemical analyses showed that all fibrillins are globally expressed in the same organs, with a number of differences on the tissue level in cartilage, perichondrium and developing bronchi. These results suggest that fibrillin-3, compared to the other fibrillins, fulfills both overlapping and distinct functions in human development.
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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.