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FBN1 encodes a member of the fibrillin family. Additionally we are shipping Fibrillin 1 Kits (42) and Fibrillin 1 Proteins (20) and many more products for this protein.
Showing 10 out of 147 products:
Cow (Bovine) Monoclonal Fibrillin 1 Primary Antibody for IEM, IHC (fro) - ABIN356183
Sakai, Keene, Engvall: Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibrils. in The Journal of cell biology 1987
Show all 3 references for ABIN356183
Cow (Bovine) Monoclonal Fibrillin 1 Primary Antibody for IEM, IHC (fro) - ABIN356181
Wright, McDaniels, Swasdison, Accavitti, Mayne, Mayne: Immunization with undenatured bovine zonular fibrils results in monoclonal antibodies to fibrillin. in Matrix biology : journal of the International Society for Matrix Biology 1994
Show all 3 references for ABIN356181
Cow (Bovine) Monoclonal Fibrillin 1 Primary Antibody for IHC (fro), IF - ABIN356182
Maier, McDaniels, Mayne: Fibrillin and elastin networks in extrafusal tissue and muscle spindles of bovine extraocular muscles. in Investigative ophthalmology & visual science 1994
Show all 3 references for ABIN356182
Cow (Bovine) Polyclonal Fibrillin 1 Primary Antibody for WB - ABIN2780329
Uyeda, Takahashi, Eto, Sato, Xu, Kanezaki, Toki, Yonesaka, Ito: Three novel mutations of the fibrillin-1 gene and ten single nucleotide polymorphisms of the fibrillin-3 gene in Marfan syndrome patients. in Journal of human genetics 2004
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 (show FBN2 Antibodies) expression with the onset of tropoelastin (show ELN Antibodies) production is consistent with a role in elastic fiber assembly.
a calcium-binding epidermal growth factor (show EGF Antibodies)-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 (show TGFB1 Antibodies) binding proteins affecting TGFbeta (show TGFB1 Antibodies) bioavailability in the ovary.
The R2726W FBN1 variant is associated with skeletal features of Marfan syndrome.
New insights into the structure, assembly and biological roles of 10-12 nm connective tissue microfibrils from fibrillin-1 studies.
Also, expansion of the mutation spectrum in FBN1 will be helpful in genetic counselling for Chinese patients with STAAD.
Data suggest that fibrillin-1 and ATP1B3 (show ATP1B3 Antibodies) are binding partners of BST-2 (show BST2 Antibodies); fibrillin-1 (unlike ATP1B3 (show ATP1B3 Antibodies)) restricts of HIV-1 replication in a mechanism independent of BST-2 (show BST2 Antibodies).
Data suggest that MFAP4 (microfibrillar-associated protein 4 (show MFAP4 Antibodies)) binds tropoelastin (show ELN Antibodies), fibrillin-1/-2, and elastin (show ELN Antibodies) cross-linking amino acid desmosine; MFAP4 (show MFAP4 Antibodies) co-localizes with fibrillin-1-positive fibers; MFAP4 (show MFAP4 Antibodies) promotes tropoelastin (show ELN Antibodies) self-assembly.
Patients with a FBN1 premature termination codon mutation had a more severe musculoskeletal phenotype than patients with an inframe mutation, suggesting the involvement of TGF-beta (show TGFB1 Antibodies) signaling dysregulation in the pathophysiologic mechanisms.
These results suggest fundamental differences in the dominant pathogenic mechanisms underlying Marfan syndrome , stiff skin syndrome and the acromelic dysplasias, which give rise to TGFbeta (show TGFB1 Antibodies) dysregulation associated with these diseases.
Progressive pathological aortic root enlargement as the result of degeneration of microfibril architecture and consequential loss of extracellular matrix integrity due to fibrillin-1 (FBN1) mutations are commonly diagnosed clinical manifestations of MFS.
Left ventricular systolic dysfunction in asymptomatic Marfan syndrome patients is related to the severity of the FBN1 gene mutation.
Data indicate that abnormal fibrillin-1 (FBN1) transcripts were indicated in fibroblasts from patients with the splice site mutation c.4817-2delA and the missense mutation c.A4925G.
Fibrillin-1 mgDelta(lpn) Marfan syndrome mutation associates with preserved proteostasis and bypass of a protein disulfide isomerase (show P4HB Antibodies)-dependent quality checkpoint
fibrillin-1 deficiency is associated with relevant dysfunction of the endothelial barrier that enables adenovirus to induce vessel-harming inflammation.
the Fbn1(C1039G/+) mouse model demonstrates mild intrinsic left ventricular dysfunction
This study demonstrated that dysfunctional fibrillin-1 impairs blood-brain barrier permeability /BCSFB integrity, facilitating peripheral leukocyte infiltration, which further degrades the BBB (show ALMS1 Antibodies)/BCSFB.
The review discuss FBN1 point mutations on the structure and function of the protein associated with Marfan syndrome in affected patients impairing protein folding and traficking, secretion, proteolysis or heparin binding.
The concomitant induction of both fibrillin-1 and alpha8 integrin in a self-limited model of glomerular injury points to a protective role of the interaction of fibrillin-1 with alpha8 integrin in the glomerulus.
Data show that fibrillin-1 (FBN1) modulates bone marrow mesenchymal stem cells (BMMSCs) lineage differentiation via IL4 receptor alpha (show IL4R Antibodies)/mTOR (show FRAP1 Antibodies) protein signaling.
latent transforming growth factor-beta-1 (show TGFB1 Antibodies) binding protein-2 was prominently associated with annular fibrils containing fibrillin-1
Activation of IL-6 (show IL6 Antibodies)-STAT3 (show STAT3 Antibodies) signaling contributes to aneurysmal dilation in fibrillin-1 deficient mice through increased MMP-9 (show MMP9 Antibodies) activity, aggravating extracellular matrix degradation.
We targeted mutations in Pkd1 (show PKD1 Antibodies) and Fbn1. Double heterozygotes displayed an exacerbation of the typical Fbn1 heterozygous aortic phenotype. The basis of this genetic interaction results from upregulation of TGF-beta (show TGFB1 Antibodies) signaling caused by Pkd1 (show PKD1 Antibodies) haploinsufficiency.
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