The evolution of extracellular fibrillins and their functional domains. [PDF]
PLoS ONE, 2012 Fibrillins constitute the major backbone of multifunctional microfibrils in elastic and non-elastic extracellular matrices, and are known to interact with several binding partners including tropoelastin and integrins.
Adam Piha-Gossack +2 more
doaj +9 more sources
Role of fibrilins in human cancer: A narrative review [PDF]
Health Science Reports, 2023 Background Fibrillin is one of the extracellular matrix glycoproteins and participates in forming microfibrils found in many connective tissues. The microfibrils enable the elasticity and stretching properties of the ligaments and support connective ...
Mahsa Mahdizadehi +3 more
doaj +2 more sources
Case Report: A Novel Homozygous Missense Variant of FBN3 Supporting It Is a New Candidate Gene Causative of a Bardet–Biedl Syndrome–Like Phenotype [PDF]
Frontiers in Genetics, 2022 Fibrillin proteins are extracellular matrix glycoproteins assembling into microfibrils. FBN1, FBN2, and FBN3 encode the human fibrillins and mutations in FBN1 and FBN2 cause connective tissue disorders called fibrillinopathies, affecting cardiovascular ...
Maria Luce Genovesi +8 more
doaj +2 more sources
Fibrillin gene family and its role in plant growth, development, and abiotic stress [PDF]
Frontiers in Plant ScienceFibrillins (FBNs), highly conserved plastid lipid-associated proteins (PAPs), play a crucial role in plant physiology. These proteins, encoded by nuclear genes, are prevalent in the plastoglobules (PGs) of chloroplasts.
Ahmed H. El-Sappah +9 more
doaj +2 more sources
Transcriptome-Wide Expression Profiling in Skin Fibroblasts of Patients with Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type. [PDF]
PLoS ONE, 2016 Joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type (JHS/EDS-HT), is likely the most common systemic heritable connective tissue disorder, and is mostly recognized by generalized joint hypermobility, joint instability complications ...
Nicola Chiarelli +7 more
doaj +6 more sources
OsFBN6 Enhances Brown Spot Disease Resistance in Rice [PDF]
PlantsBrown spot (BS) is caused by necrotrophs fungi Cochliobolus miyabeanus (C. miyabeanus) which affects rainfed and upland production in rice, resulting in significant losses in yield and grain quality.
Fang-Yuan Cao +6 more
doaj +2 more sources
Poglut2/3 double knockout in mice results in neonatal lethality with reduced levels of fibrillin in lung tissues. [PDF]
J Biol ChemFibrillin microfibrils play a critical role in the formation of elastic fibers, tissue/organ development, and cardiopulmonary function. These microfibrils not only provide structural support and flexibility to tissues, but they also regulate growth ...
Neupane S +5 more
europepmc +3 more sources
Defining the Extracellular Matrix of Rhabdomyosarcoma
Frontiers in Oncology, 2021 Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood with a propensity to metastasize. Current treatment for patients with RMS includes conventional systemic chemotherapy, radiation therapy, and surgical resection; nevertheless ...
Xiaolei Lian +17 more
doaj +1 more source
Fibrillin-1 mutations causing Weill-Marchesani syndrome and acromicric and geleophysic dysplasias disrupt heparan sulfate interactions. [PDF]
PLoS ONE, 2012 The extracellular glycoprotein fibrillin-1 forms microfibrils that act as the template for elastic fibers. Most mutations in fibrillin-1 cause Marfan syndrome with severe cardiovascular and ocular symptoms, and tall stature.
Stuart A Cain +4 more
doaj +1 more source
LTBP-2 Has a Single High-Affinity Binding Site for FGF-2 and Blocks FGF-2-Induced Cell Proliferation. [PDF]
PLoS ONE, 2015 Latent transforming growth factor-beta-1 binding protein-2 (LTBP-2) belongs to the fibrillin-LTBP superfamily of extracellular matrix proteins. LTBPs and fibrillins are involved in the sequestration and storage of latent growth factors, particularly ...
Clementine Menz +6 more
doaj +1 more source