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抗Human Fibrillin 1 抗体:
抗Mouse (Murine) Fibrillin 1 抗体:
抗Rat (Rattus) Fibrillin 1 抗体:
Human Polyclonal Fibrillin 1 Primary Antibody for IHC, IHC (p) - ABIN4311653
Zhang, Ota, Shridhar, Chien, Wu, Kuang: Network-based survival analysis reveals subnetwork signatures for predicting outcomes of ovarian cancer treatment. in PLoS computational biology 2013
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Cow (Bovine) Monoclonal Fibrillin 1 Primary Antibody for ICC, IF - ABIN259623
Shin, Lee, Kim, Kum, Jung, Park: Anti-glycation activities of phenolic constituents from Silybum marianum (Milk Thistle) flower in vitro and on human explants. in Molecules (Basel, Switzerland) 2015
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Cow (Bovine) Polyclonal Fibrillin 1 Primary Antibody for ELISA - ABIN4272120
Morisaki, Akutsu, Ogino, Kondo, Yamanaka, Tsutsumi, Yoshimuta, Okajima, Matsuda, Minatoya, Sasaki, Tanaka, Ishibashi-Ueda, Morisaki: Mutation of ACTA2 gene as an important cause of familial and nonfamilial nonsyndromatic thoracic aortic aneurysm and/or dissection (TAAD). in Human mutation 2009
Data suggest that genetic fibrillin-1 deficiency could alter normal endothelial signaling.
In cases of vascular calcification, the decreased expression of FBN1 may be partially responsible for decreased vascular elasticity and also for the decreased formation of new elastic fibers.
The coordinate upregulation of fibrillin-1 and fibrillin-2 (显示 FBN2 抗体) expression with the onset of tropoelastin (显示 ELN 抗体) production is consistent with a role in elastic fiber assembly.
a calcium-binding epidermal growth factor (显示 EGF 抗体)-like domain of fibrillin-1 c.3598G > A, p.E1200K mutation is responsible for a bovine model of Marfan syndrome
This is the first study to investigate the expression and localization of fibrillin proteins and latent TGF-beta (显示 TGFB1 抗体) binding proteins affecting TGFbeta (显示 TGFB1 抗体) bioavailability in the ovary.
This study has confirmed or corrected the clinical diagnosis, and enlarged the mutation spectrum of FBN1 and TGFBR2 (显示 TGFBR2 抗体) and confirmed that parental mosaicism may be the cause of the varied phenotypic expression of these connective tissue disorders. The results should be helpful for prenatal diagnosis and genetic counseling.
Novel FBN1 mutation (p.Cys2672Arg) has been described in a family with inherited Marfan Syndrome.
A heterozygous mutation c.5284G > A (p.Gly1762Ser) in FBN1 gene was identified in all individuals affected with acromelic dysplasia in three families.
Results demonstrate that the deleterious mutations in FBN1 largely contribute to pathogenesis of sporadic non-syndromic AD, which expands our knowledge of FBN1 variants and the genetic basis and pathology of AD.
deletions in FBN1 results in variable phenotypes of Marfan syndrome
Although causation has not been proven by this report, it certainly raises interest in a mechanistic relationship between FBN1 and left ventricular non-compaction cardiomyopathy.
Most individuals meeting these criteria have a pathogenic variant in FBN1, usually unique or observed rarely. Individuals with EL alone may also have FBN1 pathogenic variants, and the risk for aortic disease is not well known. We identified a unique cohort of 31 individuals (mean age 29, range 2-78) from nine families ascertained by a proband with EL alone, who had the same FBN1 p.R650C variant
A missense mutation (c.G6953A:p.C2318Y) and a nonsense mutation (c.C4786T:p.R1596X) were identified in the fibrillin 1 gene.
Aortic stiffness is increased in Marfan syndrome, independently from fibrillin-1 genotype.
The overexpression of miR (显示 MLXIP 抗体)-133b and silence of FBN1 could inhibit the cell proliferative, migratory and invasive abilities of gastric cancer cells, while the influence of down-regulated miR (显示 MLXIP 抗体)-133b expression and up-regulated FBN1 expression were quite the contrary.
These results suggest that the establishment of the DNA methylation (显示 HELLS 抗体) pattern within the FBN1 CpG island shore occurs after the blastocyst stage, likely during organogenesis. In conclusion, Hypo-allele ratios of the FBN1 CpG island shore correlated with FBN1 expression levels in porcine tissues.
The abundance of elastic fibers was reduced and fragmented in obesity, suggesting that the reduction in elastic fibers is initially caused by increased neprilysin (显示 MME 抗体) and decreased fibrillin-1 expression, which may inhibit formation and stabilization of elastic fibers, resulting in skin fragility in obesity.
fibrillin-1 contributes little to lipid storage and metabolic homeostasis, which is in contrast to the obesity and metabolic changes associated with MAGP1 (显示 MFAP2 抗体) deficiency.
In young Fbn1(C1039G/+) mice, aortopathy develops in the absence of detectable alterations in smooth muscle cell (SMC (显示 DYM 抗体)) TGF-beta (显示 TGFB1 抗体) signaling. Loss of physiologic SMC (显示 DYM 抗体) TGF-beta (显示 TGFB1 抗体) signaling exacerbates MFS-associated aortopathy. Our data support a protective role for SMC (显示 DYM 抗体) TGF-beta (显示 TGFB1 抗体) signaling during early development of MFS-associated aortopathy.
Studies of Marfan syndrome and congenital contractural arachnodactyly mice have correlated the skeletal phenotypes of these mutant animals with distinct pathophysiological mechanisms that reflect the contextual contribution of fibrillin-1 and -2 scaffolds to TGFbeta and BMP signaling during bone patterning, growth and metabolism. [review]
Findings demonstrate that loss of fibrillin-1 in the mouse's marrow causes significant hematopoietic abnormalities, such as hematopoietic stem cell (HSC (显示 FUT1 抗体)) depletion and augmented erythropoiesis (polycythemia). Furthermore, the distinct outcomes of systemic TGFbeta (显示 TGFB1 抗体) neutralization in mutant mice strongly suggest that fibrillin-1 differentially modulates TGFbeta (显示 TGFB1 抗体) signaling within HSC (显示 FUT1 抗体) and erythroid niches.
This study shows that Raman microspectroscopy is able to reveal structural changes in fibrillin-1 microfibrils and elastic fiber networks and to discriminate between normal and diseased networks in vivo and in vitro.
Resveratrol inhibits aortic root dilatation in Fbn1C1039G/+ Marfan mice by promoting elastin (显示 ELN 抗体) integrity and smooth muscle cell survival, involving downregulation of the aneurysm-related micro-RNA-29b in the aorta.
These results suggest that both fibrillin-1 and fibrillin-2 (显示 FBN2 抗体) expression is required to form thick oxytalan fibers in periodontal ligament.
findings show that fibrillin-1 regulates MSC (显示 MSC 抗体) activity by modulating TGFbeta (显示 TGFB1 抗体) bioavailability within the microenvironment of marrow niches.
Fibrillin-1 mgDelta(lpn) Marfan syndrome mutation associates with preserved proteostasis and bypass of a protein disulfide isomerase (显示 P4HB 抗体)-dependent quality checkpoint
the generation of a rabbit Marfanoid-progeroid-lipodystrophy model with C-terminal truncation of fibrillin-1 using a CRISPR/Cas9 system, is reported.
This gene encodes a member of the fibrillin family. The encoded protein is a large, extracellular matrix glycoprotein that serve as a structural component of 10-12 nm calcium-binding microfibrils. These microfibrils provide force bearing structural support in elastic and nonelastic connective tissue throughout the body. Mutations in this gene are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome.
, fibrillin 15
, fibrillin 1 (Marfan syndrome)
, tight skin