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 |
|
•
•
•
•
•
|
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: |
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