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Search results 1 to 100 out of 663 for Fbn1

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Type Details Score
Gene
Type: gene
Organism: human
Gene
Type: gene
Organism: zebrafish
Gene
Type: gene
Organism: macaque, rhesus
Gene
Type: gene
Organism: frog, western clawed
Gene
Type: gene
Organism: rat
Gene
Type: gene
Organism: chimpanzee
Gene
Type: gene
Organism: dog, domestic
Gene
Type: gene
Organism: cattle
Gene
Type: gene
Organism: chicken
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Gene
Type: gene
Organism: human
Publication
First Author: Goldstein C
Year: 1994
Journal: Mamm Genome
Title: Of mice and Marfan: genetic linkage analyses of the fibrillin genes, Fbn1 and Fbn2, in the mouse genome.
Volume: 5
Issue: 11
Pages: 696-700
Publication
First Author: Cook JR
Year: 2012
Journal: Genesis
Title: Generation of Fbn1 conditional null mice implicates the extracellular microfibrils in osteoprogenitor recruitment.
Volume: 50
Issue: 8
Pages: 635-41
Publication
First Author: Le Goff C
Year: 2011
Journal: Am J Hum Genet
Title: Mutations in the TGFβ binding-protein-like domain 5 of FBN1 are responsible for acromicric and geleophysic dysplasias.
Volume: 89
Issue: 1
Pages: 7-14
Publication
First Author: Davis MR
Year: 2016
Journal: Mol Genet Metab
Title: Expression of FBN1 during adipogenesis: Relevance to the lipodystrophy phenotype in Marfan syndrome and related conditions.
Volume: 119
Issue: 1-2
Pages: 174-85
Publication
First Author: Lima BL
Year: 2010
Journal: PLoS One
Title: A new mouse model for marfan syndrome presents phenotypic variability associated with the genetic background and overall levels of Fbn1 expression.
Volume: 5
Issue: 11
Pages: e14136
Publication  
First Author: Tarraf SA
Year: 2024
Journal: Front Cardiovasc Med
Title: The Fbn1 gene variant governs passive ascending aortic mechanics in the mgΔ(lpn) mouse model of Marfan syndrome when superimposed to perlecan haploinsufficiency.
Volume: 11
Pages: 1319164
GXD Expression
Probe: MGI:3053401
Assay Type: Immunohistochemistry
Annotation Date: 2022-02-21
Strength: Present
Sex: Not Specified
Emaps: EMAPS:3567624
Pattern: Not Specified
Stage: TS24
Assay Id: MGI:6874605
Age: embryonic day 16.5
Image: 4H/I
Note: Expression of Fbn1 co-localized with Thsd4 in the perichondrium.
Specimen Label: 4H/I
Detected: true
Specimen Num: 1
Publication
First Author: Siracusa LD
Year: 1996
Journal: Genome Res
Title: A tandem duplication within the fibrillin 1 gene is associated with the mouse tight skin mutation.
Volume: 6
Issue: 4
Pages: 300-13
Protein
Organism: Mus musculus/domesticus
Length: 213  
Fragment?: true
Protein
Organism: Mus musculus/domesticus
Length: 272  
Fragment?: true
Publication
First Author: Bona CA
Year: 1997
Journal: DNA Res
Title: Structure of the mutant fibrillin-1 gene in the tight skin (TSK) mouse.
Volume: 4
Issue: 4
Pages: 267-71
Publication
First Author: Jimenez SA
Year: 1986
Journal: J Biol Chem
Title: Increased collagen biosynthesis and increased expression of type I and type III procollagen genes in tight skin (TSK) mouse fibroblasts.
Volume: 261
Issue: 2
Pages: 657-62
Publication
First Author: Wu HJ
Year: 2020
Journal: Invest Ophthalmol Vis Sci
Title: Increased Susceptibility to Glaucomatous Damage in Microfibril Deficient Mice.
Volume: 61
Issue: 10
Pages: 28
Publication
First Author: Gardi C
Year: 1990
Journal: Exp Mol Pathol
Title: Serum antielastase deficiency in tight-skin mice with genetic emphysema.
Volume: 52
Issue: 1
Pages: 46-53
Publication
First Author: Szapiel SV
Year: 1981
Journal: Am Rev Respir Dis
Title: Hereditary emphysema in the tight-skin (Tsk/+) mouse.
Volume: 123
Issue: 6
Pages: 680-5
Publication  
First Author: Wu HJ
Year: 2018
Journal: eNeuro
Title: Enlarged Optic Nerve Axons and Reduced Visual Function in Mice with Defective Microfibrils.
Volume: 5
Issue: 5
Publication
First Author: Bashey RI
Year: 1993
Journal: Cardiovasc Res
Title: Increased collagen synthesis and increased content of type VI collagen in myocardium of tight skin mice.
Volume: 27
Issue: 6
Pages: 1061-5
Publication
First Author: Menton DN
Year: 1980
Journal: J Invest Dermatol
Title: The ultrastructure of collagen in the dermis of tight-skin (Tsk) mutant mice.
Volume: 74
Issue: 3
Pages: 139-47
Publication
First Author: Bocchieri MH
Year: 1993
Journal: J Autoimmun
Title: Immunological characterization of (tight skin/NZB)F1 hybrid mice with connective tissue and autoimmune features resembling human systemic sclerosis.
Volume: 6
Issue: 3
Pages: 337-51
Publication
First Author: Bona C
Year: 1994
Journal: Curr Opin Immunol
Title: Autoantibodies in scleroderma and tightskin mice.
Volume: 6
Issue: 6
Pages: 931-7
Publication
First Author: Kasturi KN
Year: 1993
Journal: Mol Immunol
Title: Tight-skin mouse autoantibody repertoire: analysis of VH and VK gene usage.
Volume: 30
Issue: 11
Pages: 969-78
Publication
First Author: Akita M
Year: 1992
Journal: Histol Histopathol
Title: Electron microscopic observations of elastic fibres in the lung and aorta of tight-skin and beta-aminopropionitrile-fed mice.
Volume: 7
Issue: 1
Pages: 39-45
Publication
First Author: Levi-Schaffer F
Year: 1996
Journal: J Invest Dermatol
Title: Inhibition of collagen synthesis and changes in skin morphology in murine graft-versus-host disease and tight skin mice: effect of halofuginone.
Volume: 106
Issue: 1
Pages: 84-8
Publication
First Author: Saito S
Year: 1999
Journal: Mol Immunol
Title: Characterization of mutated protein encoded by partially duplicated fibrillin-1 gene in tight skin (TSK) mice.
Volume: 36
Issue: 3
Pages: 169-76
Publication
First Author: Martorana PA
Year: 1989
Journal: Am Rev Respir Dis
Title: A 16-month study of the development of genetic emphysema in tight-skin mice.
Volume: 139
Issue: 1
Pages: 226-32
Publication
First Author: Ross SC
Year: 1983
Journal: Arthritis Rheum
Title: Glycosaminoglycan content in skin of the tight-skin mouse.
Volume: 26
Issue: 5
Pages: 653-7
Publication
First Author: Yao J
Year: 1992
Journal: Cardiovasc Res
Title: Decreased collagen mRNA and regression of cardiac fibrosis in the ventricular myocardium of the tight skin mouse following thyroid hormone treatment.
Volume: 26
Issue: 6
Pages: 603-7
Publication
First Author: Toyama T
Year: 2016
Journal: J Invest Dermatol
Title: Tamibarotene Ameliorates Bleomycin-Induced Dermal Fibrosis by Modulating Phenotypes of Fibroblasts, Endothelial Cells, and Immune Cells.
Volume: 136
Issue: 2
Pages: 387-98
Publication
First Author: Pablos JL
Year: 1997
Journal: J Histochem Cytochem
Title: Apoptosis and proliferation of fibroblasts during postnatal skin development and scleroderma in the tight-skin mouse.
Volume: 45
Issue: 5
Pages: 711-9
Publication
First Author: Walker M
Year: 1985
Journal: Proc Soc Exp Biol Med
Title: Mast cells and their degranulation in the Tsk mouse model of scleroderma.
Volume: 180
Issue: 2
Pages: 323-8
Publication
First Author: Pablos JL
Year: 1995
Journal: Lab Invest
Title: Transforming growth factor-beta 1 and collagen gene expression during postnatal skin development and fibrosis in the tight-skin mouse.
Volume: 72
Issue: 6
Pages: 670-8
Publication
First Author: O'dell NL
Year: 1996
Journal: Arch Oral Biol
Title: Morphological and biochemical studies of the elastic fibres in the craniomandibular joint articular disc of the tight-skin mouse.
Volume: 41
Issue: 5
Pages: 431-7
Publication
First Author: Shibata S
Year: 1993
Journal: J Clin Invest
Title: Immunochemical and molecular characterization of anti-RNA polymerase I autoantibodies produced by tight skin mouse.
Volume: 92
Issue: 2
Pages: 984-92
Publication
First Author: Tsuji-Yamada J
Year: 2001
Journal: Biochem Biophys Res Commun
Title: Effect of IL-12 encoding plasmid administration on tight-skin mouse.
Volume: 280
Issue: 3
Pages: 707-12
Publication
First Author: Rossi GA
Year: 1984
Journal: Am Rev Respir Dis
Title: Hereditary emphysema in the tight-skin mouse. Evaluation of pathogenesis.
Volume: 129
Issue: 5
Pages: 850-5
Publication
First Author: Menon RP
Year: 2006
Journal: Exp Cell Res
Title: Hammerhead ribozyme-mediated silencing of the mutant fibrillin-1 of tight skin mouse: insight into the functional role of mutant fibrillin-1.
Volume: 312
Issue: 9
Pages: 1463-74
Publication
First Author: Gardi C
Year: 1994
Journal: Exp Mol Pathol
Title: Cardiac collagen changes during the development of right ventricular hypertrophy in tight-skin mice with emphysema.
Volume: 60
Issue: 2
Pages: 100-7
Publication
First Author: Wang HW
Year: 2005
Journal: Exp Dermatol
Title: Mast cell accumulation and cytokine expression in the tight skin mouse model of scleroderma.
Volume: 14
Issue: 4
Pages: 295-302
Publication
First Author: Kasturi KN
Year: 1994
Journal: Proc Natl Acad Sci U S A
Title: Molecular characterization of J558 genes encoding tight-skin mouse autoantibodies: identical heavy-chain variable genes code for antibodies with different specificities.
Volume: 91
Issue: 17
Pages: 8067-71
Publication
First Author: DeLustro FA
Year: 1983
Journal: Cell Immunol
Title: Delayed-type hypersensitivity to elastase-soluble lung peptides in the tight-skin (Tsk) mouse.
Volume: 81
Issue: 1
Pages: 175-9
Publication
First Author: Longhurst PA
Year: 1992
Journal: J Urol
Title: Urinary bladder function in the tight-skin mouse.
Volume: 148
Issue: 5
Pages: 1611-4
Publication
First Author: Xu H
Year: 2011
Journal: Am J Physiol Cell Physiol
Title: Abnormal fibrillin-1 expression and chronic oxidative stress mediate endothelial mesenchymal transition in a murine model of systemic sclerosis.
Volume: 300
Issue: 3
Pages: C550-6
Publication  
First Author: Rossi GA
Year: 1980
Journal: Bull Eur Physiopathol Respir
Title: The tight-skin mouse: an animal model of inherited emphysema.
Volume: 16 Suppl
Pages: 157-66
Publication
First Author: Podowski M
Year: 2009
Journal: Am J Pathol
Title: Complex integration of matrix, oxidative stress, and apoptosis in genetic emphysema.
Volume: 175
Issue: 1
Pages: 84-96
Publication
First Author: Philips N
Year: 1995
Journal: J Biol Chem
Title: Increased alpha 1(I) procollagen gene expression in tight skin (TSK) mice myocardial fibroblasts is due to a reduced interaction of a negative regulatory sequence with AP-1 transcription factor.
Volume: 270
Issue: 16
Pages: 9313-21
Publication  
First Author: Green MC
Year: 1973
Journal: Mouse News Lett
Title: Tight skin (Tsk)
Volume: 48
Pages: 34
Publication
First Author: Kielty CM
Year: 1998
Journal: J Cell Biol
Title: The Tight skin mouse: demonstration of mutant fibrillin-1 production and assembly into abnormal microfibrils.
Volume: 140
Issue: 5
Pages: 1159-66
Publication
First Author: Shen L
Year: 2023
Journal: Arthritis Res Ther
Title: Iguratimod attenuated fibrosis in systemic sclerosis via targeting early growth response 1 expression.
Volume: 25
Issue: 1
Pages: 151
Publication  
First Author: Li B
Year: 2017
Journal: Bone Res
Title: Development of spinal deformities in the tight-skin mouse.
Volume: 5
Pages: 16053
Publication
First Author: Kasturi KN
Year: 1994
Journal: Int Rev Immunol
Title: Tight-skin mouse an experimental model for scleroderma.
Volume: 11
Issue: 3
Pages: 253-71
Publication
First Author: Shen Y
Year: 2005
Journal: J Invest Dermatol
Title: CpG oligodeoxynucleotides prevent the development of scleroderma-like syndrome in tight-skin mice by stimulating a Th1 immune response.
Volume: 124
Issue: 6
Pages: 1141-8
Publication
First Author: Omens JH
Year: 1994
Journal: Am J Physiol
Title: Passive ventricular mechanics in tight-skin mice.
Volume: 266
Issue: 3 Pt 2
Pages: H1169-76
Publication
First Author: Baxter RM
Year: 2005
Journal: Lab Invest
Title: Analysis of the tight skin (Tsk1/+) mouse as a model for testing antifibrotic agents.
Volume: 85
Issue: 10
Pages: 1199-209
Publication
First Author: Gardi C
Year: 1992
Journal: Exp Mol Pathol
Title: Lung collagen synthesis and deposition in tight-skin mice with genetic emphysema.
Volume: 56
Issue: 2
Pages: 163-72
Publication
First Author: Frondoza C
Year: 1996
Journal: J Autoimmun
Title: Silicone does not potentiate development of the scleroderma-like syndrome in tight skin (TSK/+) mice.
Volume: 9
Issue: 4
Pages: 473-83
Publication
First Author: Kiso Y
Year: 1992
Journal: Cell Tissue Res
Title: Granulated metrial gland cells in the pregnant uterus of mice expressing the collagen mutation tight-skin (Tsk/+).
Volume: 268
Issue: 2
Pages: 393-6
Publication
First Author: Honda N
Year: 2013
Journal: Am J Pathol
Title: miR-150 down-regulation contributes to the constitutive type I collagen overexpression in scleroderma dermal fibroblasts via the induction of integrin β3.
Volume: 182
Issue: 1
Pages: 206-16
Publication
First Author: Muryoi T
Year: 1992
Journal: Cell Immunol
Title: Self-reactive repertoire of tight skin (TSK/+) mouse: immunochemical and molecular characterization of anti-cellular autoantibodies.
Volume: 144
Issue: 1
Pages: 43-54
Publication
First Author: Ong VH
Year: 2003
Journal: Arthritis Rheum
Title: Monocyte chemoattractant protein 3 as a mediator of fibrosis: Overexpression in systemic sclerosis and the type 1 tight-skin mouse.
Volume: 48
Issue: 7
Pages: 1979-91
Publication
First Author: Saito E
Year: 2002
Journal: J Clin Invest
Title: CD19-dependent B lymphocyte signaling thresholds influence skin fibrosis and autoimmunity in the tight-skin mouse.
Volume: 109
Issue: 11
Pages: 1453-62
Publication
First Author: Weihrauch D
Year: 2016
Journal: PLoS One
Title: An IRF5 Decoy Peptide Reduces Myocardial Inflammation and Fibrosis and Improves Endothelial Cell Function in Tight-Skin Mice.
Volume: 11
Issue: 4
Pages: e0151999
Publication
First Author: Siracusa LD
Year: 1998
Journal: Mamm Genome
Title: The mouse tight skin (Tsk) phenotype is not dependent on the presence of mature T and B lymphocytes.
Volume: 9
Issue: 11
Pages: 907-9
Publication
First Author: Kasturi KN
Year: 1997
Journal: J Autoimmun
Title: B-cell deficiency does not abrogate development of cutaneous hyperplasia in mice inheriting the defective fibrillin-1 gene.
Volume: 10
Issue: 6
Pages: 505-17
Publication
First Author: Green MC
Year: 1976
Journal: Am J Pathol
Title: Tight-skin, a new mutation of the mouse causing excessive growth of connective tissue and skeleton.
Volume: 82
Issue: 3
Pages: 493-512
Publication
First Author: Gayraud B
Year: 2000
Journal: J Cell Biol
Title: New insights into the assembly of extracellular microfibrils from the analysis of the fibrillin 1 mutation in the tight skin mouse.
Volume: 150
Issue: 3
Pages: 667-80
Publication
First Author: Kodera T
Year: 2002
Journal: Proc Natl Acad Sci U S A
Title: Disrupting the IL-4 gene rescues mice homozygous for the tight-skin mutation from embryonic death and diminishes TGF-beta production by fibroblasts.
Volume: 99
Issue: 6
Pages: 3800-5
Publication
First Author: Dodig TD
Year: 2001
Journal: Arthritis Rheum
Title: Development of the tight-skin phenotype in immune-deficient mice.
Volume: 44
Issue: 3
Pages: 723-7
Publication
First Author: Marie I
Year: 2002
Journal: J Invest Dermatol
Title: Endothelial dysfunction in murine model of systemic sclerosis: tight-skin mice 1.
Volume: 119
Issue: 6
Pages: 1379-87
Publication
First Author: Kakizoe E
Year: 2001
Journal: J Invest Dermatol
Title: Isoform-selective upregulation of mast cell chymase in the development of skin fibrosis in scleroderma model mice.
Volume: 116
Issue: 1
Pages: 118-23
Publication
First Author: Granchelli JA
Year: 1995
Journal: J Neurol Sci
Title: Duchenne-like myopathy in double-mutant mdx mice expressing exaggerated mast cell activity.
Volume: 131
Issue: 1
Pages: 1-7
Publication
First Author: Phelps RG
Year: 1993
Journal: J Autoimmun
Title: Induction of skin fibrosis and autoantibodies by infusion of immunocompetent cells from tight skin mice into C57BL/6 Pa/Pa mice.
Volume: 6
Issue: 6
Pages: 701-18
Publication
First Author: Dimitriu-Bona A
Year: 1993
Journal: Cell Immunol
Title: Studies of the cellular immune response to heparan sulfate proteoglycan in the tight skin mouse.
Volume: 150
Issue: 2
Pages: 321-32
Publication
First Author: Ito S
Year: 2006
Journal: Am J Respir Cell Mol Biol
Title: Early emphysema in the tight skin and pallid mice: roles of microfibril-associated glycoproteins, collagen, and mechanical forces.
Volume: 34
Issue: 6
Pages: 688-94
Publication
First Author: Weihrauch D
Year: 2007
Journal: Am J Physiol Heart Circ Physiol
Title: Effects of D-4F on vasodilation, oxidative stress, angiostatin, myocardial inflammation, and angiogenic potential in tight-skin mice.
Volume: 293
Issue: 3
Pages: H1432-41
Publication
First Author: Matsushita T
Year: 2007
Journal: J Invest Dermatol
Title: BAFF antagonist attenuates the development of skin fibrosis in tight-skin mice.
Volume: 127
Issue: 12
Pages: 2772-80
Publication
First Author: Alexander JJ
Year: 2008
Journal: Neurochem Int
Title: TNF is a key mediator of septic encephalopathy acting through its receptor, TNF receptor-1.
Volume: 52
Issue: 3
Pages: 447-56
Publication
First Author: Lemaire R
Year: 2004
Journal: J Invest Dermatol
Title: Fibulin-2 and fibulin-5 alterations in tsk mice associated with disorganized hypodermal elastic fibers and skin tethering.
Volume: 123
Issue: 6
Pages: 1063-9
Publication
First Author: Pedroza M
Year: 2017
Journal: PLoS One
Title: Targeting of cadherin-11 decreases skin fibrosis in the tight skin-1 mouse model.
Volume: 12
Issue: 11
Pages: e0187109
Publication
First Author: Lemaire R
Year: 2010
Journal: J Invest Dermatol
Title: Antagonistic effect of the matricellular signaling protein CCN3 on TGF-beta- and Wnt-mediated fibrillinogenesis in systemic sclerosis and Marfan syndrome.
Volume: 130
Issue: 6
Pages: 1514-23
Publication  
First Author: Jones W
Year: 2019
Journal: Dis Model Mech
Title: Targeted deletion of fibrillin-1 in the mouse eye results in ectopia lentis and other ocular phenotypes associated with Marfan syndrome.
Volume: 12
Issue: 1
Publication
First Author: Avouac J
Year: 2020
Journal: J Invest Dermatol
Title: Estrogens Counteract the Profibrotic Effects of TGF-β and their Inhibition Exacerbates Experimental Dermal Fibrosis.
Volume: 140
Issue: 3
Pages: 593-601.e7
Publication
First Author: Wang W
Year: 2022
Journal: Nat Commun
Title: Fibroblast A20 governs fibrosis susceptibility and its repression by DREAM promotes fibrosis in multiple organs.
Volume: 13
Issue: 1
Pages: 6358
Publication
First Author: Asano N
Year: 2004
Journal: Am J Pathol
Title: B Lymphocyte signaling established by the CD19/CD22 loop regulates autoimmunity in the tight-skin mouse.
Volume: 165
Issue: 2
Pages: 641-50
Publication
First Author: Hasegawa M
Year: 2006
Journal: Am J Pathol
Title: B-lymphocyte depletion reduces skin fibrosis and autoimmunity in the tight-skin mouse model for systemic sclerosis.
Volume: 169
Issue: 3
Pages: 954-66
Publication
First Author: Gerdes Gyuricza I
Year: 2020
Journal: Eur J Hum Genet
Title: Is HSPG2 a modifier gene for Marfan syndrome?
Volume: 28
Issue: 9
Pages: 1292-1296
Publication
First Author: Wallace VA
Year: 1994
Journal: Eur J Immunol
Title: A role for CD4+ T cells in the pathogenesis of skin fibrosis in tight skin mice.
Volume: 24
Issue: 6
Pages: 1463-6
Publication
First Author: Samuel CS
Year: 2003
Journal: Arch Biochem Biophys
Title: Relaxin regulates fibrillin 2, but not fibrillin 1, mRNA and protein expression by human dermal fibroblasts and murine fetal skin.
Volume: 411
Issue: 1
Pages: 47-55
Publication
First Author: Chen C
Year: 2014
Journal: EMBO Mol Med
Title: Telomerase governs immunomodulatory properties of mesenchymal stem cells by regulating FAS ligand expression.
Volume: 6
Issue: 3
Pages: 322-34
Publication
First Author: Bayle J
Year: 2008
Journal: J Invest Dermatol
Title: Increased expression of Wnt2 and SFRP4 in Tsk mouse skin: role of Wnt signaling in altered dermal fibrillin deposition and systemic sclerosis.
Volume: 128
Issue: 4
Pages: 871-81