Fibrillins

A family of extracellular matrix glycoproteins that is structurally similar to LATENT TGF-BETA BINDING PROTEINS, but contain additional TGF-beta binding domains, in addition to unique domains at their N and C-terminals. Fibrillins assemble into 10-12 nm MICROFIBRILS that function in a variety of cell interactions with the EXTRACELLULAR MATRIX and developmental processes such as ELASTIC TISSUE maintenance and assembly, and the targeting of growth factors to the extracellular matrix.

Also Known As:

Networked: 297 relevant articles (2 outcomes, 27 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Reinhardt, Dieter P: 10 articles (01/2018 - 01/2005)
2.	Hubmacher, Dirk: 8 articles (01/2019 - 05/2008)
3.	Kielty, Cay M: 7 articles (10/2016 - 02/2002)
4.	Sakai, Lynn Y: 6 articles (01/2019 - 01/2005)
5.	Apte, Suneel S: 5 articles (01/2019 - 05/2015)
6.	Ramirez, Francesco: 5 articles (05/2016 - 06/2010)
7.	Cain, Stuart A: 4 articles (01/2019 - 10/2006)
8.	Lafyatis, Robert: 4 articles (04/2008 - 11/2002)
9.	Shuttleworth, C Adrian: 4 articles (10/2006 - 02/2002)
10.	Boileau, C: 4 articles (11/2003 - 01/2000)

Related Diseases

1.	Sarcoma (Soft Tissue Sarcoma) 01/01/2021 - "These results complement recent studies investigating whether soft tissue sarcomas utilize collagens, fibrillins, and collagen-modifying enzymes to alter the structural integrity of surrounding host extracellular matrix/collagen quaternary structure resulting in improved ability to improve the ability to invade regionally and metastasize, for which therapeutic targeting is possible." 01/01/2021 - "With focus on the RMS/undifferentiated pleomorphic sarcoma (UPS) continuum, we have constructed tissue microarrays of 48 murine and four human sarcomas to analyze expression of seven different collagens, fibrillins, and collagen-modifying proteins, with cross-correlation to RNA deep sequencing. "
2.	Cicatrix (Scar) 07/01/1990 - "Spatial arrangements of microfibrils in myocardial scars: application of antibody to fibrillin." 09/01/1997 - "In summary, these data indicate that ageing is associated with increased expression of fibrillin and elastin during acute wound healing and concomitant restoration of the papillary dermal architecture with an improved quality of scarring." 02/01/2012 - "However, hyaluronic acid, dermatopontin and decorin are decreased, and the expression and localisation of fibrillin and elastin fibres in the dermis are altered compared with normal skin and scars. " 05/01/2001 - "The distribution of collagens I and III, elastin and fibrillin are altered in lichen sclerosus and this is likely to contribute to the fragility, scarring and atrophy seen clinically in lichen sclerosus."
3.	Marfan Syndrome (Marfan's Syndrome) 11/01/2006 - "This review discusses mutant-fibrillin mouse models of Marfan syndrome and SSc (Tsk mice), and studies suggesting that alterations in microfibrils might contribute to human SSc. " 11/01/1997 - "Badly engineered fibrillin lessons from molecular studies of marfan syndrome." 09/01/1996 - "Herein we report a systematic study of fibrillin assembly in normal and Marfan's syndrome fibroblasts and correlations between pulse-chase, immunofluorescence, and immunoelectron microscopic data. " 09/01/1996 - "Dermal fibroblast culture as a model system for studies of fibrillin assembly and pathogenetic mechanisms: defects in distinct groups of individuals with Marfan's syndrome." 05/01/1995 - "Dermal fibroblasts from a 13-yr-old boy with isolated skeletal features of the Marfan syndrome were used to study fibrillin synthesis and processing. "
4.	Congenital contractural arachnodactyly 09/01/2004 - "Further studies are needed to elucidate the relation between fibrillin abnormality and ocular complications in Beals syndrome." 11/01/1996 - "Genetic linkage studies have linked congenital contractural arachnodactyly (CCA), a usually mild heritable connective-tissue disorder, to FBN2, the fibrillin gene on chromosome 5. Recently, FBN2 mutations in two patients with CCA have been described. " 03/01/1995 - "The gene was found to be located on chromosome 5q32-q33.2, near the locus 5q21-q31 reported for the fibrillin gene, FBN2, which has been linked to congenital contractural arachnodactyly. " 12/01/1993 - "Previous studies have mapped the human genes for two fibrillins to chromosome bands 15q21 (FBN1) and 5q23-q31 (FBN2) and have demonstrated that FBN1 mutations are associated with Marfan syndrome, while FBN2 is linked to the gene for congenital contractural arachnodactyly. " 01/01/2020 - "Mutations in fibrillins give rise to heritable connective tissue disorders, including Marfan syndrome and congenital contractural arachnodactyly. "
5.	Ectopia Lentis 02/01/1994 - "Ectopia lentis (EL), a dominantly inherited connective tissue disorder, has been genetically linked to the fibrillin gene on chromosome 15 (FBN1) in earlier studies. " 02/01/1994 - "A novel mutation of the fibrillin gene causing ectopia lentis." 09/01/1992 - "More recently, a similar association with the fibrillin gene has also been established in the dominantly inherited form of ectopia lentis. " 01/01/1995 - "We have found abnormal fibrillin microfibrils isolated from tissues and cell cultures from two cousins with Marfan syndrome whose major clinical abnormality is bilateral ectopia lentis, but who also have skeletal involvement but no cardiovascular defects. " 10/15/1994 - "Simple ectopia lentis (EL) was studied in a large family, by clinical examination and analysis of linkage to markers in the region of FBN1, the gene for fibrillin which causes Marfan syndrome on chromosome 15. "

Related Drugs and Biologics

1.	Collagen
2.	Complement System Proteins (Complement)
3.	Elastin
4.	Enzymes
5.	Fibrillin-1
6.	Proteins (Proteins, Gene)
7.	microfibrillar protein
8.	Glycoproteins (Glycoprotein)
9.	Extracellular Matrix Proteins
10.	Decorin

Related Therapies and Procedures

1.	Lasers (Laser)