ReviewJournal of muscle research and cell motility2002
Fibrillin-rich microfibrils: elastic biopolymers of the extracellular matrix.
Review in Journal of muscle research and cell motility, 2002. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
29 citing papers in PubMed, 73 citations in OpenAlex.
- Identification and evolutionary analysis of insect fibrillin with their closely related family proteins.PeerJ · 2026Article
- Degradation of Elastic Fiber Triggers Lacrimal Gland Dysfunction in Marfan Syndrome Mice.Investigative ophthalmology & visual science · 2025Article
- Screening and expression validation of key proteins for secondary hair follicle growth in cashmere goats based on iTRAQ quantitative proteomics technology.Frontiers in veterinary science · 2024Article
- Case report: Biochemical and clinical phenotypes caused by cysteine substitutions in the epidermal growth factor-like domains of fibrillin-1.Frontiers in genetics · 2022Article
- Article
- Fell-Muir Lecture: Fibrillin microfibrils: structural tensometers of elastic tissues?International journal of experimental pathology · 2017Review
- Comprehensive proteomic characterization of stem cell-derived extracellular matrices.Biomaterials · 2017Article
- Vesicoureteral reflux and the extracellular matrix connection.Pediatric nephrology (Berlin, Germany) · 2017Review
- Stromal Modulators of TGF-β in Cancer.Journal of clinical medicine · 2017Review
- Lysyl oxidase-like 1 polymorphisms in a southwestern Greek cataract population with pseudoexfoliation syndrome.Clinical ophthalmology (Auckland, N.Z.) · 2016Article
- Intrinsic cardiomyopathy in Marfan syndrome: results from in-vivo and ex-vivo studies of the Fbn1C1039G/+ model and longitudinal findings in humans.Pediatric research · 2015Article
- Localized micro- and nano-scale remodelling in the diabetic aorta.Acta biomaterialia · 2014Article
- Extracellular matrix synthesis in vascular disease: hypertension, and atherosclerosis.Journal of biomedical research · 2014Review
- Association study of common variations of FBN1 gene and essential hypertension in Han Chinese population.Molecular biology reports · 2014Article
- Elastogenesis at the onset of human cardiac valve development.Development (Cambridge, England) · 2013Article
- The complexity of elastic fibre biogenesis in the skin--a perspective to the clinical heterogeneity of cutis laxa.Experimental dermatology · 2013Article
- Comparative immunolocalisation of fibrillin-1 and perlecan in the human foetal, and HS-deficient hspg2 exon 3 null mutant mouse intervertebral disc.Histochemistry and cell biology · 2013Article
- Pseudoexfoliation syndrome, a systemic disorder with ocular manifestations.Human genomics · 2012Review
- Colocalization in vivo and association in vitro of perlecan and elastin.Histochemistry and cell biology · 2011Article
- Patient-to-patient variability in autologous pericardial matrix scaffolds for cardiac repair.Journal of cardiovascular translational research · 2011Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Fibrillin-rich microfibrils are evolutionarily ancient macromolecular assemblies of the extracellular matrix. They have unique extensible properties that endow vascular and other tissues with long-range elasticity. Microfibril extensibility supports the low pressure closed circulations of lower organisms such as crustaceans. In higher vertebrates, microfibrils act as a template for elastin deposition and are components of mature elastic fibres. In man, the importance of microfibrils is highlighted by the linkage of mutations in their principal structural component, fibrillin-1, to the heritable disease Marfan syndrome which is characterised by severe cardiovascular, skeletal and ocular defects. When isolated from tissues, fibrillin-rich microfibrils have a complex ultrastructural organisation with a characteristic 'beads-on-a-strong' appearance. X-ray fibre diffraction studies and biomechanical testing have shown that microfibrils are reversibly extensible at tissue extensions of 100%. Ultrastructural analysis and 3D reconstructions of isolated microfibrils using automated electron tomography have revealed new details of how fibrillin molecules are aligned within microfibrils in untensioned and extended states, and delineated the role of calcium in regulating microfibril beaded periodicity, rest length and molecular organisation. The molecular basis of how fibrillin molecules assemble into microfibrils, the central role of cells in regulating this process, and the identity of other molecules that may coassemble into microfibrils are now being elucidated. This information will enhance our understanding of the elastic mechanism of these unique extracellular matrix polymers, and may lead to new microfibril-based strategies for repairing elastic tissues in ageing and disease.
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12785107W2131224625What OpenQuestion holds
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.