Relevant bibliographies by topics / FBN1

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Academic literature on the topic 'FBN1'

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Published: 4 June 2021
Last updated: 4 February 2022

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Journal articles on the topic "FBN1"

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Bastian, Nicole A., Rosemary A. Bayne, Katja Hummitzsch, Nicholas Hatzirodos, Wendy M. Bonner, Monica D. Hartanti, Helen F. Irving-Rodgers, Richard A. Anderson, and Raymond J. Rodgers. "Regulation of fibrillins and modulators of TGFβ in fetal bovine and human ovaries." Reproduction 152, no. 2 (August 2016): 127–37. http://dx.doi.org/10.1530/rep-16-0172.

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Fibrillins 1–3 are stromal extracellular matrix proteins that play important roles in regulating TGFβ activity, which stimulates fibroblasts to proliferate and synthesize collagen. In the developing ovary, the action of stroma is initially necessary for the formation of ovigerous cords and subsequently for the formation of follicles and the surface epithelium of the ovary. FBN3 is highly expressed only in early ovarian development and then it declines. In contrast, FBN1 and 2 are upregulated in later ovarian development. We examined the expression of FBN1–3 in bovine and human fetal ovaries. We used cell dispersion and monolayer culture, cell passaging and tissue culture. Cells were treated with growth factors, hormones or inhibitors to assess the regulation of expression of FBN1–3. When bovine fetal ovarian tissue was cultured, FBN3 expression declined significantly. Treatment with TGFβ-1 increased FBN1 and FBN2 expression in bovine fibroblasts, but did not affect FBN3 expression. Additionally, in cultures of human fetal ovarian fibroblasts (9–17weeks gestational age), the expression of FBN1 and FBN2 increased with passage, whereas FBN3 dramatically decreased. Treatment with activin A and a TGFβ family signaling inhibitor, SB431542, differentially regulated the expression of a range of modulators of TGFβ signaling and of other growth factors in cultured human fetal ovarian fibroblasts suggesting that TGFβ signaling is differentially involved in the regulation of ovarian fibroblasts. Additionally, since the changes in FBN1–3 expression that occur in vitro are those that occur with increasing gestational age in vivo, we suggest that the fetal ovarian fibroblasts mature in vitro.
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Nishimura, Akira, Haruya Sakai, Shiro Ikegawa, Hiroshi Kitoh, Nobuyuki Haga, Satoshi Ishikiriyama, Toshiro Nagai, et al. "FBN2,FBN1,TGFBR1, andTGFBR2 analyses in congenital contractural arachnodactyly." American Journal of Medical Genetics Part A 143A, no. 7 (2007): 694–98. http://dx.doi.org/10.1002/ajmg.a.31639.

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Wang, Zanxin, Xianmian Zhuang, Bailang Chen, Junmin Wen, Fang Peng, Xiling Liu, and Minxin Wei. "99-Case Study of Sporadic Aortic Dissection by Whole Exome Sequencing Indicated Novel Disease-Associated Genes and Variants in Chinese Population." BioMed Research International 2020 (October 2, 2020): 1–12. http://dx.doi.org/10.1155/2020/7857043.

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Background. In this study, the whole exome sequencing in human aortic dissection, a highly lethal cardiovascular disease, was investigated to explore the aortic dissection-associated genes and variants in Chinese population. Methods. Whole exome sequencing was performed in 99 cases of aortic dissection. All single nucleotide polymorphisms (SNPs), insertions/deletions (InDels), and copy number variations (CNVs) were filtered to exclude the benign variants. Enrichment analysis and disease-gene correlation analysis were performed. Results. 3425873 SNPs, 685245 InDels, and 1177 CNVs were identified, and aortic dissection-associated SNPs, InDels, and CNVs were collected. After the disease correlation analysis, 20 candidate genes were identified. Part of these genes such as MYH11, FBN1, and ACTA2 were consistent with previous studies, while MLX, DAB2IP, EP300, ZFYVE9, PML, and PRKCD were newly identified as candidate aortic dissection-associated genes. Conclusion. The pathogenic and likely pathogenic variants in most of AD-associated genes (FBN1, MYH11, EFEMP2, TGFBR2, FBN2, COL3A1, and MYLK) were identified in our cohort study, and pathogenic CNVs involved in MYH11, COL family, and FBN were also identified which are not detectable by other NGS analysis. The correlation between MLX, DAB2IP, EP300, ZFYVE9, PML, PRKCD, and aortic dissection was identified, and EP300 may play a key role in AD.
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Zhang, H., SD Apfelroth, W. Hu, EC Davis, C. Sanguineti, J. Bonadio, RP Mecham, and F. Ramirez. "Structure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matrices." Journal of Cell Biology 124, no. 5 (March 1, 1994): 855–63. http://dx.doi.org/10.1083/jcb.124.5.855.

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During the previous cloning of the fibrillin gene (FBN1), we isolated a partial cDNA coding for a fibrillin-like peptide and mapped the corresponding gene (FBN2) to human chromosome 5. (Lee, B., M. Godfrey, E. Vitale, H. Hori, M. G. Mattei, M. Sarfarazi, P. Tsipouras, F. Ramirez, and D. W. Hollister. 1991. Nature [Lond.]. 352:330-334). The study left, however, unresolved whether or not the FBN2 gene product is an extracellular component structurally related to fibrillin. Work presented in this report clarifies this important point. Determination of the entire primary structure of the FBN2 gene product demonstrated that this polypeptide is highly homologous to fibrillin. Immunoelectron microscopy localized both fibrillin proteins to elastin-associated extracellular microfibrils. Finally, immunohistochemistry revealed that the fibrillins co-distribute in elastic and non-elastic connective tissues of the developing embryo, with preferential accumulation of the FBN2 gene product in elastic fiber-rich matrices. These results support the original hypothesis that the fibrillins may have distinct but related functions in the formation and maintenance of extracellular microfibrils. Accordingly, we propose to classify the FBN1 and FBN2 gene products as a new family of extracellular proteins and to name its members fibrillin-1 and fibrillin-2, respectively.
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Chen, Chider, Kentaro Akiyama, Dandan Wang, Xingtian Xu, Bei Li, Alireza Moshaverinia, Frank Brombacher, Lingyun Sun, and Songtao Shi. "mTOR inhibition rescues osteopenia in mice with systemic sclerosis." Journal of Experimental Medicine 212, no. 1 (December 22, 2014): 73–91. http://dx.doi.org/10.1084/jem.20140643.

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Fibrillin-1 (FBN1) deficiency-induced systemic sclerosis is attributed to elevation of interleukin-4 (IL4) and TGF-β, but the mechanism underlying FBN1 deficiency–associated osteopenia is not fully understood. We show that bone marrow mesenchymal stem cells (BMMSCs) from FBN1-deficient (Fbn1+/−) mice exhibit decreased osteogenic differentiation and increased adipogenic differentiation. Mechanistically, this lineage alteration is regulated by IL4/IL4Rα-mediated activation of mTOR signaling to down-regulate RUNX2 and up-regulate PPARγ2, respectively, via P70 ribosomal S6 protein kinase (P70S6K). Additionally, we reveal that activation of TGF-β/SMAD3/SP1 signaling results in enhancement of SP1 binding to the IL4Rα promoter to synergistically activate mTOR pathway in Fbn1+/− BMMSCs. Blockage of mTOR signaling by osteoblastic-specific knockout or rapamycin treatment rescues osteopenia phenotype in Fbn1+/− mice by improving osteogenic differentiation of BMMSCs. Collectively, this study identifies a previously unrecognized role of the FBN1/TGF-β/IL4Rα/mTOR cascade in BMMSC lineage selection and provides experimental evidence that rapamycin treatment may provide an anabolic therapy for osteopenia in Fbn1+/− mice.
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Maylem, Excel Rio, Leon Spicer, Isadora Batalha, and Luis Schutz. "PSIV-5 Developmental and hormonal regulation of gene expression of fibrillin-1 (FBN1) and the asprosin receptor, olfactory receptor family 4 subfamily M member 1 (OR4M1), in bovine ovarian cells." Journal of Animal Science 98, Supplement_4 (November 3, 2020): 283–84. http://dx.doi.org/10.1093/jas/skaa278.510.

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Abstract Asprosin is a novel fasting-induced protein associated with insulin resistance and polycystic ovaries in humans. It is encoded by FBN1 gene and produced when FBN1 is cleaved by the enzyme furin. In cattle, the role of asprosin is unknown. To characterize mRNA abundance of FBN1, furin, and the asprosin receptor, OR4M1, in granulosa (GC) and theca cells (TC), and identify hormones regulating FBN1 mRNA expression, GC and TC from small (< 6 mm; SM) and large (>5 mm; LG) follicles were collected from heifers at an abattoir and used for real-time gene expression analysis or in vitro evaluation of hormone regulation. SMTC had 151-fold greater (P < 0.05) FBN1 mRNA abundance than SMGC, and LGTC had 50-fold greater (P < 0.05) FBN1 mRNA than LGGC. In contrast, OR4M1 mRNA abundance was significantly greater (by 81-fold) in SMGC than LGGC and did not differ from SMTC, but LGTC had 9-fold greater (P < 0.05) OR4M1 mRNA abundance than LGGC. Furin mRNA was significantly greater (by 2.6-fold) in SMGC than SMTC but did not differ between LGTC and LGGC. In SMGC, leptin, insulin, GH, FSH, EGF, and steroids had no effect (P >0.10) on FBN1 mRNA abundance. In contrast, TGFB1, WNT3A and FGF9 increased (P < 0.05) and IGF1 significantly decreased SMGC FBN1 mRNA abundance. In LGTC, leptin, insulin, LH, IGF1 and steroids did not significantly affect FBN1 mRNA, but TGFB1, WNT3A, EGF, FGF2 and FGF9 increased (P< 0.05) FBN1 mRNA abundance. Altogether, FBN1 mRNA was more highly expressed in TC than GC and was stimulated by TGFB1, WNT3A and FGF9 in both cell types. Developmental and hormonal regulation of FBN1, furin and OR4M1 along with a greater expression of OR4M1 mRNA in GC than TC suggests that asprosin may be acting as a paracrine regulator of ovarian follicular function in cattle.
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Maylem, Excel Rio S., Leon J. Spicer, Isadora Batalha, and Luis F. Schutz. "Discovery of a possible role of asprosin in ovarian follicular function." Journal of Molecular Endocrinology 66, no. 1 (January 2021): 35–44. http://dx.doi.org/10.1530/jme-20-0218.

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Asprosin is a novel fasting-induced protein encoded by fibrillin-1 (FBN1) gene, produced when FBN1 is cleaved by the enzyme furin, and is associated with insulin resistance and polycystic ovarian syndrome in humans. To characterize mRNA abundance of FBN1, FURIN, and the presumed asprosin receptor, olfactory receptor family 4 subfamily M member 1 (OR4M1) in granulosa (GC) and theca cells (TC), and identify hormones regulating FBN1 mRNA expression, GC and TC from small (1–5 mm; SM) and large (>8 mm; LG) follicles were collected from ovaries of heifers obtained at an abattoir and used for real-time PCR gene expression analysis or in vitro evaluation of hormone regulation and asprosin effects. SMTC had 151-fold greater (P < 0.05) FBN1 mRNA abundance than SMGC, and LGTC had 50-fold greater FBN1 mRNA than LGGC. In contrast, OR4M1 mRNA was 81-fold greater in SMGC than LGGC and did not differ from SMTC, but LGTC had 9-fold greater OR4M1 mRNA than LGGC. FURIN mRNA was 2.6-fold greater in SMTC than SMGC, but did not differ among follicular sizes. In cultured TC, leptin, insulin, LH, IGF1 and steroids did not affect FBN1 mRNA, but TGFB1 increased (P < 0.05) FBN1 mRNA by 2.2-fold; EGF and FGFs increased FBN1 mRNA by 1.3- to 1.5-fold. Asprosin enhanced LH-induced TC androstenedione production, reduced IGF1-induced TC proliferation, and had no effect on progesterone production. Developmental regulation of FBN1, FURIN and OR4M1 along with direct effects of asprosin on TC suggests that asprosin may be a novel regulator of ovarian follicular function.
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Benarroch, Louise, Mélodie Aubart, Marie-Sylvie Gross, Marie-Paule Jacob, Pauline Arnaud, Nadine Hanna, Guillaume Jondeau, and Catherine Boileau. "Marfan Syndrome Variability: Investigation of the Roles of Sarcolipin and Calcium as Potential Transregulator of FBN1 Expression." Genes 9, no. 9 (August 21, 2018): 421. http://dx.doi.org/10.3390/genes9090421.

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Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder that displays a great clinical variability. Previous work in our laboratory showed that fibrillin-1 (FBN1) messenger RNA (mRNA) expression is a surrogate endpoint for MFS severity. Therefore, an expression quantitative trait loci (eQTL) analysis was performed to identify trans-acting regulators of FBN1 expression, and a significant signal reached genome-wide significant threshold on chromosome 11. This signal delineated a region comprising one expressed gene, SLN (encoding sarcolipin), and a single pseudogene, SNX7-ps1 (CTD-2651C21.3). We first investigated the region and then looked for association between the genes in the region and FBN1 expression. For the first time, we showed that the SLN gene is weakly expressed in skin fibroblasts. There is no direct correlation between SLN and FBN1 gene expression. We showed that calcium influx modulates FBN1 gene expression. Finally, SLN gene expression is highly correlated to that of the neighboring SNX7-ps1. We were able to confirm the impact of calcium influx on FBN1 gene expression but we could not conclude regarding the role of sarcolipin and/or the eQTL locus in this regulation.
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Lin, Mao, Sen Zhao, Gang Liu, Yingzhao Huang, Chenxi Yu, Yanxue Zhao, Lianlei Wang, et al. "Identification of novel FBN1 variations implicated in congenital scoliosis." Journal of Human Genetics 65, no. 3 (December 11, 2019): 221–30. http://dx.doi.org/10.1038/s10038-019-0698-x.

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AbstractCongenital scoliosis (CS) is a form of scoliosis caused by congenital vertebral malformations. Genetic predisposition has been demonstrated in CS. We previously reported that TBX6 loss-of-function causes CS in a compound heterozygous model; however, this model can explain only 10% of CS. Many monogenic and polygenic CS genes remain to be elucidated. In this study, we analyzed exome sequencing (ES) data of 615 Chinese CS from the Deciphering Disorders Involving Scoliosis and COmorbidities (DISCO) project. Cosegregation studies for 103 familial CS identified a novel heterozygous nonsense variant, c.2649G>A (p.Trp883Ter) in FBN1. The association between FBN1 and CS was then analyzed by extracting FBN1 variants from ES data of 574 sporadic CS and 828 controls; 30 novel variants were identified and prioritized for further analyses. A mutational burden test showed that the deleterious FBN1 variants were significantly enriched in CS subjects (OR = 3.9, P = 0.03 by Fisher’s exact test). One missense variant, c.2613A>C (p.Leu871Phe) was recurrent in two unrelated CS subjects, and in vitro functional experiments for the variant suggest that FBN1 may contribute to CS by upregulating the transforming growth factor beta (TGF-β) signaling. Our study expanded the phenotypic spectrum of FBN1, and provided nove insights into the genetic etiology of CS.
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Jensen, Sacha A., Ondine Atwa, and Penny A. Handford. "Assembly assay identifies a critical region of human fibrillin-1 required for 10–12 nm diameter microfibril biogenesis." PLOS ONE 16, no. 3 (March 18, 2021): e0248532. http://dx.doi.org/10.1371/journal.pone.0248532.

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The human FBN1 gene encodes fibrillin-1 (FBN1); the main component of the 10–12 nm diameter extracellular matrix microfibrils. Marfan syndrome (MFS) is a common inherited connective tissue disorder, caused by FBN1 mutations. It features a wide spectrum of disease severity, from mild cases to the lethal neonatal form (nMFS), that is yet to be explained at the molecular level. Mutations associated with nMFS generally affect a region of FBN1 between domains TB3-cbEGF18—the "neonatal region". To gain insight into the process of fibril assembly and increase our understanding of the mechanisms determining disease severity in MFS, we compared the secretion and assembly properties of FBN1 variants containing nMFS-associated substitutions with variants associated with milder, classical MFS (cMFS). In the majority of cases, both nMFS- and cMFS-associated neonatal region variants were secreted at levels comparable to wild type. Microfibril incorporation by the nMFS variants was greatly reduced or absent compared to the cMFS forms, however, suggesting that nMFS substitutions disrupt a previously undefined site of microfibril assembly. Additional analysis of a domain deletion variant caused by exon skipping also indicates that register in the neonatal region is likely to be critical for assembly. These data demonstrate for the first time new requirements for microfibril biogenesis and identify at least two distinct molecular mechanisms associated with disease substitutions in the TB3-cbEGF18 region; incorporation of mutant FBN1 into microfibrils changing their integral properties (cMFS) or the blocking of wild type FBN1 assembly by mutant molecules that prevents late-stage lateral assembly (nMFS).
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Dissertations / Theses on the topic "FBN1"

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Suk, Ji Young. "Molecular consequences of protein misfolding mutations in FBN1." Thesis, University of Oxford, 2003. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.270282.

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Halliday, Dorothy Jean. "Molecular analysis of FBN1 mutations in Marfan syndrome and related disorders." Thesis, University of London, 2001. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.272202.

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Hutchinson, Sarah. "Molecular analysis of the Marfan syndrome." Thesis, University of Oxford, 2000. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.343402.

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Delhon, Laure. "Rôle d’ADAMTSL2 et FBN1 dans l’ossification endochondrale : étude des modèles murins mimant la dysplasie géléophysique." Thesis, Sorbonne Paris Cité, 2017. http://www.theses.fr/2017USPCB081/document.

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La dysplasie géléophysique (DG) est une maladie rare qui appartient à la famille des dysplasies acroméliques. Cette pathologie est caractérisée par un retard statural, une brachydactylie, une raideur articulaire, une dysmorphie faciale, une peau épaisse, une atteinte bronchopulmonaire et une surcharge valvulaire cardiaque conduisant le plus souvent à une mort précoce dans les premières années de la vie. Deux modes de transmissions ont été identifiés. Le premier autosomique récessif est dû à des mutations dans le gène ADAMTSL2. Le second, autosomique dominant est dû à un hot-spot de mutations dans les exons 41 et 42 qui codent pour le domaine Transforming Growth Factor (TGF) β-binding protein-like domain 5 (TB5) du gène FBN1. FBN1 et ADAMTSL2 codent pour des protéines sécrétées de la matrice extracellulaire (MEC). FBN1 code pour la fibrilline-1, une composante des microfibrilles qui jouent un rôle dans la biodisponibilité du TGFβ. La protéine ADAMTSL2 fait partie de la famille des ADAMTS mais n’a pas d’activité enzymatique dû à l’absence de domaine catalytique. Sa fonction est encore inconnue. Cependant des partenaires d’ADAMTSL2 ont été identifiés par notre équipe : latent-transforming growth factor beta-binding protein 1 (LTBP1) et FBN1 qui sont directement impliqués dans le stockage de TGFβ. Récemment une autre protéine, FBN2, a aussi été découverte comme partenaire d’ADAMTSL2 (Hubmacher D et. al.). L’objectif de ma thèse était de comprendre le mécanisme physiopathologique de la DG, grâce à l’analyse de modèles murins. Un premier modèle murin pour la forme récessive de la DG appelé CreCMV; Adamtsl2f/f (ou KO) a été généré. L’analyse phénotypique de ces souris a montré un retard statural, des os longs courts, des extrémités courtes. Dans les plaques de croissance des os longs des souris mutantes, nous avons observé une désorganisation des colonnes chondrocytaires associée à une diminution de l’expression du collagène de type 10, marqueur de la différentiation des chondrocytes. L’analyse de la matrice extracellulaire des plaques de croissance a révélé une désorganisation structurale importante. Une diminution de la fibrilline-1 et de LTBP-1 a été observée ainsi qu’une augmentation de l’activation de la voie de signalisation TGFβ au niveau de la plaque de croissance des souris mutantes. Nous avons observé une désorganisation du réseau microfibrillaire sur des cultures de chondrocytes de souris mutantes. Ces résultats nous ont permis de suggérer que la protéine ADAMTSL2 est impliquée dans la structure du réseau microfibrillaire, lieu de stockage du TGFβ et de démontrer un rôle majeur d’ADAMTSL2 dans la régulation de la chondrogenèse. Afin d’étudier la forme dominante de la DG, le modèle FBN1TB5+/- a été généré. Il est issu d’un système knock-in avec une mutation dans l’exon 42 du gène fbn1 qui correspond au domaine TB5 de la fibrilline-1. Nos résultats ont montré une réduction de la taille des souris hétérozygotes et homozygotes en comparaison aux souris sauvages au stade P1 et P30. Au stade P1, nous avons observé des chondrocytes plus larges et une dérégulation des marqueurs de la chondrogenèse au niveau de la plaque de croissance des fémurs des souris hétérozygotes, ainsi que chez les souris homozygotes. De plus, nous avons observé une très forte mortalité des souris homozygotes vers l’âge de 2 ou 3 mois. Nous en avons conclu que des mutations domaine TB5 de la fibrilline étaient liées à un retard statural et donc que FBN1 avait un rôle majeur dans la chondrogenèse
Geleophysic dysplasia (GD) is a rare disease, which belong to acromelic group. This pathology is characterized by short stature, brachydactyly, joint stiffness, thick skin, facial dimorphism, broncho-pulmonary insufficiency and cardiac disease which lead to an early death in the first years of life. Two mode of inheritance have been identified. The first one, autosomal recessive, is due to mutations in ADAMTSL2 gene. The second, autosomal dominant, is due to hot-spot mutations in exon 41-42 of FBN1 gene, which encode the Transforming Growth Factor (TGF) β-binding protein-like domain 5 (TB5) of the protein. FBN1 and ADAMTSL2 encode secreted proteins of the extracellular matrix (ECM). FBN1 encodes fibrilline-1, component of microfibrillar network, playing a role in the bioavailability of TGF- β. ADAMTSL2 protein belongs to ADAMTS family, but does not have enzymatic activity due to lack of catalytic domain. Its function remains unknown. However, ADAMTSL2 partners have been identified by our team: latent-transforming growth factor beta-binding protein 1 (LTBP1) and FBN1, which are directly implied in storage of TGF-β. Recently, another protein, FBN2, have been identified as an ADAMTSL2 partner (Hubmacher D et. al.). The aim of my study was to understand the physiopathological mechanism of Geleophysic dysplasia by analysing murine models. A first murine model for the GD recessive form, CreCMV; Adamtsl2f/f (KO), have been generated. Phenotypic analysis of these mice showed short stature and shorter long bones and extremities. In long bone growth plate of mutant mice, we observed disorganization of chondrocyte columns, associated with decrease of collagen 10 expression, marker of chondrocyte differentiation. Analysis of ECM in growth plate revealed strong structural disorganization. Decrease of FBN1 and LTBP1 and were observed with an overactivation of TGF-β pathway in growth plate of mutant mice. We observed disorganization of microfibrillar network in chondrocyte cultures of mutant mice. These results suggest that ADAMTSL2 protein is implied in structure of microfibrillar network, where is stored TGF-β, and demonstrate major role of ADAMTSL2 in chondrogenesis. In order to study dominant form of GD, mouse model FBN1TB5+/-, have been generated. The mice were obtained by knock-in system, with mutation in exon 42 of FBN1 gene. Our results showed short stature of heterozygous (HT) and homozygous (Ho) mice compared to wild)type mice, at stage P1 and P30. At stage P1, we observed larger chondrocytes and deregulation of chondrogenesis markers in growth plate of HT and Ho mice. Furthermore, we observed high mortality of Ho mice at 2-3 months. We concluded that mutations in TB5 domain of FBN1 were linked to short stature and thus FBN1 have major role in chondrogenesis
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Ades, Lesley Carole. "The Marfan syndrome and related phenotypes : delineation of various phenotypes and analysis of the fibrillin gene (FBN1) for putative mutations /." Title page, abstract and contents only, 1995. http://web4.library.adelaide.edu.au/theses/09MD/09mda232.pdf.

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McGettrick, Aileen Jane. "Molecular consequences of mutations in FBNI." Thesis, University of Oxford, 2002. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.249501.

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Bastos, Marcelo Bratenahl. "Estudo de obtenção de revestimento de elementos combustíveis para reatores FBNR." reponame:Biblioteca Digital de Teses e Dissertações da UFRGS, 2008. http://hdl.handle.net/10183/25072.

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Este trabalho teve por objetivo obter revestimento de carbeto de silício para esferas combustíveis utilizadas em reatores nucleares do tipo FBNR, através da sinterização de SiC por reação com silício metálico (RBSiC). As matérias-primas foram moídas em moinho de bolas por 24 horas e as temperaturas utilizadas na sinterização foram de 1500° e 2000°C, durante tempos que variaram de 30 a 240 minutos. As amostras foram caracterizadas quanto a fases cristalinas, densidade, microestrutura e resistência mecânica. As peças sinterizadas a 2000°C apresentaram valores de resistência mecânica na faixa de 95 MPa, e densidade de cerca de 90% foram alcançadas, superiores aos valores encontrados para 1500°C.Foram obtidos revestimentos com as técnicas de gel casting e spin coating. A resistência mecânica desses revestimentos foi de, aproximadamente, 50% das amostras sinterizadas a 2000°C.
The aim of this work was to get covering of silicon carbide for use in nuclear fuel reactors of type FBNR, through the sintering of SiC by reaction bonded silicon carbide (RBSiC). The samples were homogenized in a ball mill and the sintering temperatures were 1500°C and 2200°C, during times that varied of 30 until 240 minutes. The product was characterized by crystalline phases, density, microstructure and mechanical resistance. The samples sintering at 2000°C had presented values of mechanical resistance around of 95 MPa, and density around 90%, better that samples sintering at 1500°C. Gel casting and Spin coating techniques had success in coverings process. The mechanical resistance of this coverings were around 50% of the samples sintering at 2000°C.
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Payne, Miranda. "Characterization of mouse embryonic stem cells deficient in Fbh1 and Blm helicases." Thesis, University of Oxford, 2010. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.526496.

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Vincent, Olivier. "Identification et caracterisation d'elements regulateurs de la transcription du gene fbp1 de saccharomyces cerevisiae." Paris 7, 1994. http://www.theses.fr/1994PA077315.

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Parmi la grande variete de sources de carbone que la levure saccharomyces cerevisiae est capable d'utiliser, le glucose occupe une place preferentielle. Il est a l'origine d'un certain nombre d'effets physiologiques chez cet organisme, qui se traduisent notamment par la repression de la transcription de genes codant pour des enzymes impliques dans le metabolisme des sources de carbone alternatives. Nous avons caracterise plusieurs elements regulateurs du promoteur du gene fbp1 qui code pour lenzyme neoglucogenique fructose-1,6-bisphosphatase et qui est reprime par le glucose. Nous avons mis en evidence l'union in vitro a certaines sequences du promoteur de ce gene de plusieurs proteines, dont le represseur mig1 qui intervient dans la repression catabolique de nombreux genes. Nous avons teste la capacite de ces regions du promoteur a activer ou reprimer la transcription d'un promoteur heterologue fusionne au gene lacz. Nous avons identifie deux elements activateurs amont du site d'union de mig1. Nous avons montre que l'ensemble des elements regulateurs de ce promoteur est controle par le glucose et par la proteine kinase cat1 qui est un element central dans le processus de repression catabolique
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Bacquin, Agathe. "Régulation de l’hélicase FBH1 et conséquences sur le maintien de la stabilité génétique chez l’homme." Thesis, Paris 11, 2012. http://www.theses.fr/2012PA11T060.

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Bien que la recombinaison homologue (RH) soit requise, notamment, pour la réparation fidèle des cassures double brins et la prise en charge des fourches de réplication bloquées, une mauvaise régulation de ce mécanisme peut provoquer des réarrangements chromosomiques importants et des pertes d’hétérozygoties. Chez la levure S. cerevisiae, la forme SUMOylée du facteur de processivité des polymérases réplicatives, PCNA, recruterait l’hélicase Srs2 au niveau des fourches de réplication bloquées afin de prévenir les évènements de RH inappropriés via la dissociation du nucléofilament de Rad51. Préalablement à ce projet, notre équipe a montré que la SUMOylation de PCNA sur la lysine 164 existe chez l’homme et qu’elle est présente en particulier dans les cellules déficientes en polymérase translésionnelle η (Pol η). Au cours de cette thèse, nous avons d’abord examiné la localisation de cette forme SUMOylée et montrons qu’elle s’accumule au niveau des dommages induits par une irradiation aux ultra-violets (UV), ce qui suggère son implication dans la réponse à ce type de lésions.Dans le but de préciser la fonction de cette forme modifiée, nous nous sommes demandé si celle-ci était impliquée dans le recrutement d’une hélicase anti-recombinogène.L’hélicase humaine FBH1 a été proposée récemment comme homologue fonctionnel potentiel de Srs2 : elle complémente partiellement les levures déficientes en Srs2, possède une activité anti-recombinogène et s’accumule aux sites de cassures double brins ou de stress réplicatif. Afin de caractériser plus précisément la fonction et le mode de régulation de l’hélicase FBH1 dans les cellules humaines, nous avons examiné sa localisation subcellulaire en l’absence de dommage et après traitement aux UV et à un agent méthylant l’ADN. Nous montrons que FBH1 est recrutée au niveau des foyers de réplication où elle est colocalisée avec PCNA. Après traitement génotoxique, FBH1 s’accumule aux sites de dommages de l’ADN de façon précoce et transitoire. Nous montrons que PCNA contrôle l’accumulation de FBH1 pendant la réplication et en réponse à des dommages via une interaction directe par le biais de deux motifs distincts d’interaction à PCNA : PIP et APIM. FBH1 n’interagit cependant pas de façon préférentielle avec PCNA-SUMO.De plus, nous montrons que le recrutement de FBH1 est suivi de sa polyubiquitination et de sa dégradation par la voie du protéasome. Cette dégradation dépend de l’action conjuguée de PCNA et du complexe ubiquitine-ligase CRL4Cdt2. Elle est nécessaire au recrutement optimal de Pol η.Notre hypothèse est donc que FBH1 serait recrutée sur l’ADN via une interaction avec PCNA au moment de la réplication ou en réponse à un stress génotoxique, afin de limiter les événements de recombinaison non programmés dépendant de RAD51. Par la suite, PCNA et CRL4Cdt2 provoqueraient la dégradation de FBH1 afin de limiter le temps de résidence de l’hélicase qui pourrait interférer avec la prise en charge des dommages par le mécanisme de synthèse translésionnelle
Although Homologous Recombination (HR) is required for error-free repair of double-strand breaks and stalled or collapsed replication forks, it has to be highly regulated to prevent unscheduled genome rearrangements and loss of heterozygosity. In yeast S. cerevisiae, the SUMOylated form of Proliferating Cell Nuclear Antigen (PCNA) recruits the DNA helicase Srs2 at stalled replication forks to prevent unscheduled HR events by disrupting Rad51 nucleoprotein. In our laboratory, previous results showed that PCNA is also SUMOylated in human on lysine 164, especially in translesion polymerase η (Pol η) deficient cells.During my phD, I first studied the localization of SUMO-PCNA and showed that it accumulates at UV-induced DNA damage. It suggests that PCNA is involved in the DNA damage response to this kind of lesions. To characterize the function of this modified form of PCNA, we wondered whether it could recruit an anti-recombinogenic helicase.The human FBH1 helicase was recently thought to act as a functional homolog of Srs2, since it can partially complement Srs2-deficient S. cerevisiae strains. Besides, hFBH1 has an anti-recombinogenic activity and accumulates at sites of DNA breaks or replication stress.To further characterize the function and regulation of hFBH1 in human cells, we examined its subcellular localization in response to several DNA damaging agents. Our results showed that, without external treatment, FBH1 accumulates into replication foci where it colocalizes with PCNA. After genotoxic treatment, FBH1 accumulates early ant transiently to DNA damage. We show that PCNA coordinates the accumulation of FBH1 during replication and after DNA damage through direct interaction via two distinct PCNA interaction motifs: PIP and APIM. However, FBH1 does not interact preferentially with SUMO-PCNA.We also show that FBH1 recruitment is followed by its polyubiquitination and degradation by the proteasome. This degradation depends on PCNA and the ubiquitin-ligase CRL4Cdt2 and is required for Pol η proper recruitment to UV-induced DNA damage. These findings suggest that PCNA recruits FBH1 at stalled replication forks or in response to DNA damage to limit unscheduled RAD51-dependent recombination. Then, PCNA and CRL4Cdt2 would promote FBH1 degradation to enable translesion synthesis
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Books on the topic "FBN1"

1

Phylactou, Leonidas Andreas. Antisense hammerhead ribozymes: Application to cleavage and down regulation of expression of FBN1 mRNA. Birmingham: University of Birmingham, 1995.

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FBN: Federal Bureau Narcotics : a novel. Wayzata, Minn: Library Publication Corp., 1988.

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Price, Susan. Genetic bone and joint disease. Edited by Patrick Davey and David Sprigings. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199568741.003.0276.

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Genetic conditions affecting the skeleton and supporting structures are individually rare and heterogeneous. This chapter presents an approach to assessing patients with suspected skeletal dysplasia, osteogenesis imperfecta, Marfan syndrome, and Ehlers–Danlos syndrome. Skeletal dysplasias are caused by abnormalities of bone growth and modelling; the commonest non-lethal type is achondroplasia, with an incidence of 1/10 000 to 1/30 000. The typical presentation of osteogenesis imperfecta is with multiple fractures, sometimes prenatally. There may be associated short stature, bone deformity, dentogenesis imperfecta, blue sclera, and hearing loss. Most patients with osteogenesis imperfecta have mutations in COL1A1 or COL1A2. Marfan syndrome is a connective tissue disease with a pattern of symptoms related to the presence of fibrillin in tissues. Typically, affected individuals are of tall, thin stature, with long fingers and toes (arachnodactyly), a pectus deformity, and scoliosis. Between 66% and 91% of individuals with Marfan syndrome have a mutation in fibrillin-1 (FBN1; locus: 15q21). All forms of Ehlers–Danlos syndrome present with variable thinning and fragility of skin, leading to easy bruising and poor scar formation. There is skin and joint laxity. In severe forms, blood vessels and internal organs are affected.
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25 Years Fbn. Publications Intl, 2007.

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International, Publications. Best Loved Fbn Recipes. Publications International, 2007.

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Leonard, Michael D. Fbn: Federal Bureau of Narcotics. Library Pubns Corp, 1988.

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Torre Hernández, María Eugenia de la. Caracterización bioquímica y molecular de la actividad de nucleasa inducida por la micotoxina fumonisina B1 (FB1) en embriones de maíz durante la germinación. Universidad Nacional Autónoma de México, Facultad de Química - Dirección General de Publicaciones y Fomento Editorial, 2015. http://dx.doi.org/10.22201/dgpyfe.9786070270703e.2015.

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Nigeria, First Bank of, MBC International Bank, and FBN (Merchant Bankers), eds. Scheme of merger: (under section 100 of the Investments and Securities Act No. 45, 1999) between First Bank of Nigeria Plc and MBC International Bank Plc and FBN (Merchant Bankers) Limited. [Nigeria: s.n., 2005.

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Book chapters on the topic "FBN1"

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De Paepe, Anne, Bart Loeys, and Paul Coucke. "Mutation Analysis of the FBN1 Gene in Individuals with Marfan Syndrome: Sensitivity, Methods, Clinical Indications." In Marfan Syndrome: A Primer for Clinicians and Scientists, 93–100. Boston, MA: Springer US, 2004. http://dx.doi.org/10.1007/978-1-4419-9013-6_8.

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Parniangtong, Sathit. "Strategic Sourcing: Fact-Based Negotiation (FBN)." In Management for Professionals, 65–79. Singapore: Springer Singapore, 2016. http://dx.doi.org/10.1007/978-981-10-1723-0_6.

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Kim, Paul, and Günter Krause. "Local Bijective Gabriel Correspondence and Torsion Theoretic FBN Rings." In Advances in Ring Theory, 175–89. Boston, MA: Birkhäuser Boston, 1997. http://dx.doi.org/10.1007/978-1-4612-1978-1_15.

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Liu, Ruohao, Ning Zhong, Xiaofei Zhang, Yang Yang, and Jiajin Huang. "Classification of Mental Arithmetic Cognitive States Based on CNN and FBN." In Brain Informatics, 220–30. Cham: Springer International Publishing, 2019. http://dx.doi.org/10.1007/978-3-030-37078-7_22.

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Mair, H., C. Detter, H. G. Klein, U. Grau, A. Welz, and B. Reichart. "Molekulargenetischer Nachweis von Mutationen im Fibrillin 1 (FBN 1) Gen bei klinisch identifizierten Marfanpatienten." In Klinik und Forschung in der Chirurgie unter dem Aspekt von Effizienz und Ökonomie, 1455–56. Berlin, Heidelberg: Springer Berlin Heidelberg, 1997. http://dx.doi.org/10.1007/978-3-642-60881-0_430.

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"Marfan Syndrome (MFS, FBN1)." In Encyclopedia of Genetics, Genomics, Proteomics and Informatics, 1154–55. Dordrecht: Springer Netherlands, 2008. http://dx.doi.org/10.1007/978-1-4020-6754-9_9876.

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Goff, Carine Le, and Valérie Cormier-Daire. "ADAMTS10, ADAMTS17, and FBN1: The Weill-Marchesani Syndrome." In Epstein's Inborn Errors of Development, 1335–37. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199934522.003.0203.

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Schepers, Dorien, and Bart Loeys. "Genetics of vascular disease: Marfan syndrome and aortic disease." In ESC CardioMed, 713–15. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198784906.003.0160.

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Marfan syndrome is an autosomal dominant, multisystemic disorder, presenting with skeletal, ocular, and cardiovascular symptoms. This connective tissue disease is caused by mutations in FBN1, encoding fibrillin-1, which is an important extracellular matrix protein. Marfan syndrome shows significant clinical overlap with Loeys–Dietz syndrome, which is caused by genetic defects in components of the transforming growth factor-beta pathway: TGFBR1, TGFBR2, TGFB2, TGFB3, SMAD2, and SMAD3. Overlapping clinical features between Marfan syndrome and Loeys–Dietz syndrome include aortic root aneurysm, arachnodactyly, scoliosis, and pectus deformity.
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Schepers, Dorien, and Bart Loeys. "Genetics of vascular disease: Marfan syndrome and aortic disease." In ESC CardioMed, 713–15. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198784906.003.0160_update_001.

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Marfan syndrome is an autosomal dominant, multisystemic disorder, presenting with skeletal, ocular, and cardiovascular symptoms. This connective tissue disease is caused by mutations in FBN1, encoding fibrillin-1, which is an important extracellular matrix protein. Marfan syndrome shows significant clinical overlap with Loeys–Dietz syndrome, which is caused by genetic defects in components of the transforming growth factor-beta pathway: TGFBR1, TGFBR2, TGFB2, TGFB3, SMAD2, and SMAD3. Overlapping clinical features between Marfan syndrome and Loeys–Dietz syndrome include aortic root aneurysm, arachnodactyly, scoliosis, and pectus deformity.
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"Fbl1." In Encyclopedia of Cancer, 1700. Berlin, Heidelberg: Springer Berlin Heidelberg, 2016. http://dx.doi.org/10.1007/978-3-662-46875-3_100891.

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Conference papers on the topic "FBN1"

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Lu, Hong, and Lu Lu. "Expression quantitative trait loci and genetic regulatory network analysis of Fbn1." In INTERNATIONAL SYMPOSIUM ON THE FRONTIERS OF BIOTECHNOLOGY AND BIOENGINEERING (FBB 2019). AIP Publishing, 2019. http://dx.doi.org/10.1063/1.5110812.

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"FB1-4.pdf." In 2007 Conference on Lasers and Electro Optics and the Pacific Rim Conference on Lasers and Electro-Optics. IEEE, 2007. http://dx.doi.org/10.1109/cleopr.2007.4391092.

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Guo, Peng, Hong Ma, Ruizhi Chen, Pin Li, Shaolin Xie, and Donglin Wang. "FBNA: A Fully Binarized Neural Network Accelerator." In 2018 28th International Conference on Field Programmable Logic and Applications (FPL). IEEE, 2018. http://dx.doi.org/10.1109/fpl.2018.00016.

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"Session FB1 system level architecture exploration." In 2009 IEEE International SOC Conference (SOCC). IEEE, 2009. http://dx.doi.org/10.1109/soccon.2009.5398011.

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Sefidvash, Farhang. "Preliminary Evaluation of the Fixed Bed Nuclear Reactor Concept Using IAEA-INPRO Methodology." In 12th International Conference on Nuclear Engineering. ASMEDC, 2004. http://dx.doi.org/10.1115/icone12-49405.

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The Atomic Energy Agency (IAEA) through its INPRO Project has developed a methodology to evaluate the innovative nuclear reactors. The main objectives of INPRO are to help to ensure that nuclear energy is available in the 21st century in a sustainable manner; and bring together both technology holders and technology users to achieve desired innovations in nuclear reactors and nuclear fuel cycles which are to be acceptable to the public because they are economic, safe, proliferation resistant, sustainable, and having reduced environmental impact. Here is a preliminary application of this methodology (IAEA-TECDOC-1362) to evaluate the Fixed Bed Nuclear Reactor Concept (FBNR). Some of the characteristics of the proposed reactor are: The FBNR is based on pressurized light water reactor technology. It is a small, modular, and integrated primary circuit reactor. The fuel elements of FBNR are 8 mm diameter spherical uranium dioxide pellets cladded by zircaloy or made of compacted TRISO type fuel particles. The reactor core is suspended by the flow of water coolant. The stop in flow causes the fuel elements leave the reactor core by the force of gravity and fall into a passively cooled fuel chamber or even leave the reactor completely and become deposited in the spent fuel pool. It is an inherently safe and passively cooled reactor concept. FBNR in its advanced versions can use supercritical steam or helium gas as coolant, and utilize MOX or thorium fuel.
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Waitschat, Arne, Lennard Nordmann, Frank Thielecke, and Valérie Pommier-Budinger. "Enhanced Toolbox for the Combined Analysis of Fluid- and Structure-Borne Noise of Hydraulic Systems." In BATH/ASME 2016 Symposium on Fluid Power and Motion Control. American Society of Mechanical Engineers, 2016. http://dx.doi.org/10.1115/fpmc2016-1701.

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Today’s available software packages that feature dedicated hydraulic system noise analysis capabilities focus on the Fluid-Borne Noise (FBN) prediction in frequency or time domain. However, some noise sensitive hydraulic installation areas, like light weight structures in aviation, drive the development of an extended toolbox that also covers the prediction of Structure-Borne Noise (SBN) and Fluid-Structure-Interaction (FSI) in hydraulic systems. This conference contribution presents the concept and design of such a toolbox. It is implemented in a Matlab Simulink/ Simscape environment by FBN-, SBN- and FSI- model libraries. For a given study case a simulation model is generated using elements from these libraries. The simulation results are experimentally validated using a dedicated hydraulic system noise test rig. It features a rotary valve as FBN source and a pipe system equipped with dynamic pressure transducers for FBN detection and acceleration sensors for SBN detection. The analysis capabilities of such a toolbox are considered beneficial in particular for future (pre-/re-) design projects in the aviation environment, which hold challenging application constraints for efficient hydraulic system noise reduction devices: Besides obligatory strong limits on component weight and size, the high safety and reliability standards demand simple and maintenance-free onboard devices. Hence solutions with minimum hardware efforts are preferred. In this context, the proposed toolbox can be used for a combined tuning of type and location of standard/simple FBN silencers together with type and location of SBN effecting pipe clamps in order to optimize the overall system noise pattern towards an increased equipment durability and passenger comfort.
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Pan, Min, Beichen Ding, Chenggang Yuan, Jun Zou, and Huayong Yang. "Novel Integrated Control of Fluid-Borne Noise in Hydraulic Systems." In BATH/ASME 2018 Symposium on Fluid Power and Motion Control. American Society of Mechanical Engineers, 2018. http://dx.doi.org/10.1115/fpmc2018-8828.

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The noise in hydraulic machines presents itself as fluid-borne noise (FBN), structure-borne noise (SBN) and air-borne noise (ABN). FBN is caused by the unsteady flow produced by pumps and motors or the operation of digital hydraulics, and propagates through the system causing SBN, which in turn causes ABN. This article reports on a novel integrated FBN attenuation approach, which employs a hybrid control system by integrating an active feedforward noise attenuator with passive tuned flexible hoses. The passive hoses are tuned to cancel the high-frequency pressure pulsations, whilst the active controller is designed to attenuate the dominant harmonic ripples. Adaptive notch filters with a variable step-size filtered-X Least Mean Square algorithm were applied in the new designed active piezoelectric actuator with high preload and operating forces, a wide bandwidth and very good linear dynamics. A time-domain hose model considering coupling of longitudinal wall and fluid waves was used to model and tune the flexible hose. Very good FBN cancellation was achieved by using the proposed integrated control approach, which was validated by comparing with numerical simulation and experiments. It can be concluded that the active attenuator with passive flexible hoses can form an effective, cost-efficient and practical solution for FBN attenuation. As the problem of high noise levels generated by hydraulically powered machines has risen significantly in awareness amongst industry and the general public, this work constitutes an important contribution to the sustainable development of low noise hydraulic fluid power machines.
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Adouane, Belkacem, Guus Witteveen, Wiebren de Jong, and Jos P. van Buijtenen. "Low Fuel-NOx Combustion of Synthetic LCV Gas." In ASME Turbo Expo 2006: Power for Land, Sea, and Air. ASMEDC, 2006. http://dx.doi.org/10.1115/gt2006-90991.

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Fuel NOx is one of the main issues related to the combustion of biomass derived Low Calorific Value (LCV) Gas. The high NOx emissions accompanying the combustion of that fuel in gas turbines or gas engines are compromising the CO2 neutral character of biomass and are a barrier towards the introduction of this green energy source in the market. The reduction of NOx emissions has been one of the main preoccupations of researchers in the LCV gas combustion field. Although, much has been achieved for thermal NOx which is caused mainly by the conversion of the nitrogen of the air in high temperature regions, less work has been devoted to the reduction of fuel NOx, which has as a main source the fuel bound nitrogen FBN, namely ammonia in case of biomass. Reducing the conversion of the FBN to NOx has been the main issue in recent research work. However, fuel NOx could be reduced significantly applying methods; like washing the gas in a scrubber prior its entrance to the combustor, and SNCR or SCR methods applied at the exhaust. But those solutions stay very expensive in terms of polluted waste water and catalyst cost. In this paper, the approach is to reduce the conversion of FBN to NOx inside a newly designed combustor. The idea is to optimize the combustion process ending up with the lowest possible conversion of FBN to NOx. The LCV gas used in the experiments described in this paper is made by mixing CO, CO2, H2, natural gas and N2 with proportions comparable to those of the real LCV gas. This gas is then doped with NH3 to simulate the FBN. In this paper the conversion ratio of FBN to NOx versus the FBN concentration is presented. Furthermore, the system is investigated in terms of the effect of CH4 concentration on the conversion of FBN to NOx. And measurements along the combustor axis were performed with a traversing probe where temperature and important emissions along the axis were measured. In all the experiments described in the paper, The LCV gas has an HHV (High Calorific Value) ranging from 4 to 7Mj/nm3. The newly designed combustor contains an embedded inner cylinder. In these experiments presented are without that embedded cylinder. The purpose of the current experiments is to be compared to the later experiments with the insert in order to define clearly the effect of the inner cylinder. Furthermore, this arrangement, i.e. without the insert, gave us the opportunity to traverse the combustor by a probe and to measure temperature and species profiles, which is of a great importance in defining the key parameter controlling the conversion of NH3 to NOx.
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Adouane, Belkacem, Guus Witteveen, Weibren de Jong, and Jos P. van Buijtenen. "An Experimental Investigation of a Newly Designed Combustor for LCV Gas With Low NOx Emissions From Fuel Bound Nitrogen." In ASME Turbo Expo 2004: Power for Land, Sea, and Air. ASMEDC, 2004. http://dx.doi.org/10.1115/gt2004-54038.

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Biomass derived LCV gas represents one of the best alternatives for fossil fuels. It is very attractive, because of its neutral aspects concerning CO2 emissions. However, on the other hand, the high content of fuel bound nitrogen results in high NOx emissions. This is one of the major problems related to the application of biomass derived LCV gas in gas turbines or gas engines. Reducing the conversion of fuel bound nitrogen (FBN) to NOx has been one of the main preoccupations for researchers working in the field of LCV gas combustion. At the section Energy Technology of Delft University of Technology, a group of researchers is busy with optimizing a newly designed combustor for LCV gas and low NOx emissions. With the new design, it is expected to end up with a very low conversion of FBN to NOx by optimizing the design and combustion process. The newly designed combustor is investigated experimentally and by CFD modeling. In this paper, the experimental part is presented. In all the experiments described below, natural gas diluted with nitrogen was the simulated LCV gas and ammonia (NH3) is injected into the fuel gas to simulate the FBN. The fuel gas has an HCV (High Calorific Value) of 5MJ/mn3. The combustor shows a very important optimal regime, where a minimum in conversion of FBN to NOx is achieved while maintaining a very low CO emissions. As low as 8% conversion ratio of NH3 to NOx has been achieved at high NH3 concentration in the LCV gas (3.45vol.%), and a minimum of 30% conversion was achieved for low ammonia concentrations (3100 ppmv).
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Lee, Sungmin, Hyunwoo Kim, Hyunkoo Kang, and BuHyun Youn. "Abstract 3598: Phosphoglycerate mutase 1 inhibitor causes metabolic disadvantages in glioblastoma by rescue of FBP1 repression." In Proceedings: AACR Annual Meeting 2019; March 29-April 3, 2019; Atlanta, GA. American Association for Cancer Research, 2019. http://dx.doi.org/10.1158/1538-7445.am2019-3598.

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Reports on the topic "FBN1"

1

Babcock, Darcie. Mutation Analysis of Fibrillin-2 (FBN2) and Microfibril Associated Protein-3 (MFAP-3): Two Genes Associated with Congenital Contractural Arachnodactyly (CCA), also known as Beal's Syndrome. Portland State University Library, January 2000. http://dx.doi.org/10.15760/etd.7073.

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