Recombinant Mouse Fibrillin-1 (Fbn1), partial

1. The abundance of elastic fibers was reduced and fragmented in obesity, suggesting that the reduction in elastic fibers is initially caused by increased neprilysin and decreased fibrillin-1 expression, which may inhibit formation and stabilization of elastic fibers, resulting in skin fragility in obesity. PMID: 27892729
2. fibrillin-1 contributes little to lipid storage and metabolic homeostasis, which is in contrast to the obesity and metabolic changes associated with MAGP1 deficiency. PMID: 26902431
3. In young Fbn1(C1039G/+) mice, aortopathy develops in the absence of detectable alterations in smooth muscle cell (SMC) TGF-beta signaling. Loss of physiologic SMC TGF-beta signaling exacerbates MFS-associated aortopathy. Our data support a protective role for SMC TGF-beta signaling during early development of MFS-associated aortopathy. PMID: 28119285
4. 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] PMID: 26408953
5. Findings demonstrate that loss of fibrillin-1 in the mouse's marrow causes significant hematopoietic abnormalities, such as hematopoietic stem cell (HSC) depletion and augmented erythropoiesis (polycythemia). Furthermore, the distinct outcomes of systemic TGFbeta neutralization in mutant mice strongly suggest that fibrillin-1 differentially modulates TGFbeta signaling within HSC and erythroid niches. PMID: 26610678
6. 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. PMID: 27956365
7. Resveratrol inhibits aortic root dilatation in Fbn1C1039G/+ Marfan mice by promoting elastin integrity and smooth muscle cell survival, involving downregulation of the aneurysm-related micro-RNA-29b in the aorta. PMID: 27283746
8. These results suggest that both fibrillin-1 and fibrillin-2 expression is required to form thick oxytalan fibers in periodontal ligament. PMID: 25524144
9. findings show that fibrillin-1 regulates MSC activity by modulating TGFbeta bioavailability within the microenvironment of marrow niches. PMID: 26189658
10. Fibrillin-1 mgDelta(lpn) Marfan syndrome mutation associates with preserved proteostasis and bypass of a protein disulfide isomerase-dependent quality checkpoint PMID: 26718974
11. fibrillin-1 deficiency is associated with relevant dysfunction of the endothelial barrier that enables adenovirus to induce vessel-harming inflammation. PMID: 26840980
12. the Fbn1(C1039G/+) mouse model demonstrates mild intrinsic left ventricular dysfunction PMID: 26042521
13. This study demonstrated that dysfunctional fibrillin-1 impairs blood-brain barrier permeability /BCSFB integrity, facilitating peripheral leukocyte infiltration, which further degrades the BBB/BCSFB. PMID: 25797463
14. 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. PMID: 26281765
15. 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. PMID: 25482639
16. Data show that fibrillin-1 (FBN1) modulates bone marrow mesenchymal stem cells (BMMSCs) lineage differentiation via IL4 receptor alpha/mTOR protein signaling. PMID: 25534817
17. latent transforming growth factor-beta-1 binding protein-2 was prominently associated with annular fibrils containing fibrillin-1 PMID: 24867584
18. Activation of IL-6-STAT3 signaling contributes to aneurysmal dilation in fibrillin-1 deficient mice through increased MMP-9 activity, aggravating extracellular matrix degradation. PMID: 24449804
19. We targeted mutations in Pkd1 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 signaling caused by Pkd1 haploinsufficiency. PMID: 24071006
20. Report immunolocalization of fibrillin-1/perlecan in HS-deficient hspg2 exon 3 null mutant mouse intervertebral disc. PMID: 23104139
21. analysis of the global biomechanical and local cell-matrix deformation relationships in the aging and Marfan related Fbn1 mutated murine mitral valve PMID: 22984535
22. Data show that fibrillin-1 expression was intense in the ligamentum flavum from E16 through P0, with E17 appearing to be the stage with the greatest staining intensity. PMID: 22685574
23. ADAMTSL6beta has a role in fibrillin-1 microfibril formation PMID: 21880733
24. fibrillin-1 plays a predominantly greater role than fibrillin-2 in determining the material properties of bones PMID: 21440062
25. Data support the development of therapies based on increasing Fbn1 expression. PMID: 21152435
26. Tight-skinned (Tsk-/+) mice demonstrate abnormal "big fibrillin" expression (405 kDa)and chronic oxidative stress that contribute to mesenchymal trnasition and reduction in angiogenesis in a model of systemic sclerosis. PMID: 21160034
27. Data show that a cluster of 205 genes, including Fbn1, were selectively expressed by mouse cell lines of different mesenchymal lineages. PMID: 20551991
28. Fibrillin 1 is indispensable for normal tissue architecture and gene expression of periodontal ligaments. PMID: 20714769
29. Fibrillin-1 and -2, the structural components of extracellular microfibrils, differentially regulate TGF-beta and bone morphogenetic protein (BMP) bioavailability in bone. PMID: 20855508
30. analysis of mutant fibrillin-1 microfibrils PMID: 20529844
31. Results indicated that ADAMTSL-6 is a novel microfibril-associated protein that binds directly to fibrillin-1 and promotes fibrillin-1 matrix assembly. PMID: 19940141
32. interaction with microfibril-associated glycoprotein-2 PMID: 12122015
33. there is a molecular basis for compensation of one fibrillin (1 or 2) by the other during fetal life PMID: 12429739
34. relaxin does not regulate its mRNA and protein expression by human dermal fibroblasts and murine fetal skin. PMID: 12590922
35. mice deficient in fibrillin-1 have marked dysregulation of transforming growth factor-beta (TGF-beta) activation and signaling, resulting in apoptosis in the developing lung, in a model of Marfan syndrome PMID: 12598898
36. Tight skin fibrillin 1 altered extracellular matrix organization and caused fibrosis by affecting deposition of MAGP-2 or other fibrillin-1-associated proteins. PMID: 15022335
37. Data are consistent with a model that invokes haploinsufficiency for wild type fibrillin-1, rather than production of mutant protein, as the primary determinant of failed microfibrillar assembly in Marfan syndrome. PMID: 15254584
38. High affinity interactions between fibrillin-1 and perlecan were found by kinetic binding studies with dissociation constants in the low nanomolar range. PMID: 15657057
39. ANG II can directly induce fibrillin-1 expression in cardiac fibroblasts PMID: 15849235
40. fibrillin-1 may contribute to glomerular damage in hypertensive and diabetic kidney disease PMID: 16380460
41. involvement of fibrillin-2 in the initial assembly of the aortic matrix overlaps in part with fibrillin-1 and continued fibrillin-1 deposition is absolutely required for the maturation and function of the vessel during neonatal life PMID: 16407178
42. Results suggest that changes in microfibril architecture brought about by fibrillin-1 mutation lead to a complex disease phenotype. PMID: 16488411
43. The distribution of Fbn1 was studied in the endometrium during estrus, diestrus, and pregnancy. PMID: 17587800
44. In Tsk mice the mutation in Fbn1 alters bone cell metabolism. PMID: 17705049
45. Fibrillin-1 microfibril deposition is dependent on fibronectin assembly. PMID: 18653538
46. Loss of fibrillin-1 deposition promotes the production of intracellular reactive oxygen species and abnormal accumulation of phosphorylated TGF-beta-activated kinase 1 and p38 MAPK in Marfan syndrome phenotype. PMID: 19109253
47. fibrillin-1, but not fibulin-2 or fibulin-4, is required for appropriate matrix assembly of latent transforming growth factor beta-binding proteins PMID: 19349279
48. The promoter region of the Fbn1 gene has been characterized. PMID: 19573590
49. Fragmentation of elastic fibers leads to increased vascular stiffness, which promotes features of multifocal plaque instability in atherosclerotic apolipopotein E-deficient mice with a mutated fibrillin 1 gene. PMID: 19948973

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KEGG: mmu:14118

STRING: 10090.ENSMUSP00000028633

UniGene: Mm.271644