About   Help   FAQ
Gene Expression Literature Summary
Symbol
Name
ID
Fbn1
fibrillin 1
MGI:95489

45 matching records from 45 references.

Summary by Age and Assay: Numbers in the table indicate the number of results matching the search criteria.
Age E6.5 E8.5 E9 E9.5 E10.5 E12 E12.5 E13.5 E14.5 E15 E15.5 E16.5 E17.5 E18.5 E20 P
Immunohistochemistry (section) 1 2 5 5 1 2 3 3 1 8
In situ RNA (section) 1 1 1 1 1 1 4 2 4 3
In situ RNA (whole mount) 1 1 3 1
Northern blot 2 3
Western blot 1 1 1
RT-PCR 1 2 2 5 1 3 1 11
cDNA clones 1

Summary by Gene and Reference: Number indicates the number of results matching the search criteria recorded for each reference.
* Indicates detailed expression data entries available
Fbn1  fibrillin 1   (Synonyms: Fib-1)
Results  Reference
6J:70592 Arteaga-Solis E, Gayraud B, Lee SY, Shum L, Sakai L, Ramirez F, Regulation of limb patterning by extracellular microfibrils. J Cell Biol. 2001 Jul 23;154(2):275-81
1*J:174611 Azhar M, Brown K, Gard C, Chen H, Rajan S, Elliott DA, Stevens MV, Camenisch TD, Conway SJ, Doetschman T, Transforming growth factor Beta2 is required for valve remodeling during heart development. Dev Dyn. 2011 Sep;240(9):2127-41
2*J:135507 Bayle J, Fitch J, Jacobsen K, Kumar R, Lafyatis R, Lemaire R, Increased expression of Wnt2 and SFRP4 in Tsk mouse skin: role of Wnt signaling in altered dermal fibrillin deposition and systemic sclerosis. J Invest Dermatol. 2008 Apr;128(4):871-81
1*J:313619 Bedogni F, Hevner RF, Cell-Type-Specific Gene Expression in Developing Mouse Neocortex: Intermediate Progenitors Implicated in Axon Development. Front Mol Neurosci. 2021;14:686034
2*J:226844 Collin GB, Hubmacher D, Charette JR, Hicks WL, Stone L, Yu M, Naggert JK, Krebs MP, Peachey NS, Apte SS, Nishina PM, Disruption of murine Adamtsl4 results in zonular fiber detachment from the lens and in retinal pigment epithelium dedifferentiation. Hum Mol Genet. 2015 Dec 15;24(24):6958-74
1*J:148410 Combes AN, Lesieur E, Harley VR, Sinclair AH, Little MH, Wilhelm D, Koopman P, Three-dimensional visualization of testis cord morphogenesis, a novel tubulogenic mechanism in development. Dev Dyn. 2009 May;238(5):1033-41
2J:190996 d'Amaro R, Scheidegger R, Blumer S, Pazera P, Katsaros C, Graf D, Chiquet M, Putative functions of extracellular matrix glycoproteins in secondary palate morphogenesis. Front Physiol. 2012;3:377
2J:18222 Davis EC, Immunolocalization of microfibril and microfibril-associated proteins in the subendothelial matrix of the developing mouse aorta. J Cell Sci. 1994 Mar;107(Pt 3):727-36
2J:114987 Ge G, Greenspan DS, BMP1 controls TGFbeta1 activation via cleavage of latent TGFbeta-binding protein. J Cell Biol. 2006 Oct 9;175(1):111-20
1J:283038 Gharibeh L, Komati H, Bosse Y, Boodhwani M, Heydarpour M, Fortier M, Hassanzadeh R, Ngu J, Mathieu P, Body S, Nemer M, GATA6 Regulates Aortic Valve Remodeling, and Its Haploinsufficiency Leads to Right-Left Type Bicuspid Aortic Valve. Circulation. 2018 Sep 4;138(10):1025-1038
1*J:171409 GUDMAP Consortium, GUDMAP: the GenitoUrinary Development Molecular Anatomy Project. www.gudmap.org. 2004;
2J:280264 Hubmacher D, Taye N, Balic Z, Thacker S, Adams SM, Birk DE, Schweitzer R, Apte SS, Limb- and tendon-specific Adamtsl2 deletion identifies a role for ADAMTSL2 in tendon growth in a mouse model for geleophysic dysplasia. Matrix Biol. 2019 Sep;82:38-53
7J:221347 Hubmacher D, Wang LW, Mecham RP, Reinhardt DP, Apte SS, Adamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia - a novel mouse model providing insights into geleophysic dysplasia. Dis Model Mech. 2015 May 1;8(5):487-99
1J:296352 Imhof T, Korkmaz Y, Koch M, Sengle G, Schiavinato A, EMILIN proteins are novel extracellular constituents of the dentin-pulp complex. Sci Rep. 2020 Sep 18;10(1):15320
1*J:161299 Ito Y, Toriuchi N, Yoshitaka T, Ueno-Kudoh H, Sato T, Yokoyama S, Nishida K, Akimoto T, Takahashi M, Miyaki S, Asahara H, The Mohawk homeobox gene is a critical regulator of tendon differentiation. Proc Natl Acad Sci U S A. 2010 Jun 8;107(23):10538-42
1J:179089 Jaffe M, Sesti C, Washington IM, Du L, Dronadula N, Chin MT, Stolz DB, Davis EC, Dichek DA, Transforming Growth Factor-beta Signaling in Myogenic Cells Regulates Vascular Morphogenesis, Differentiation, and Matrix Synthesis. Arterioscler Thromb Vasc Biol. 2012 Jan;32(1):e1-e11
1J:306108 Karpinski BA, Maynard TM, Bryan CA, Yitsege G, Horvath A, Lee NH, Moody SA, LaMantia AS, Selective disruption of trigeminal sensory neurogenesis and differentiation in a mouse model of 22q11.2 deletion syndrome. Dis Model Mech. 2022 Feb 1;15(2):dmm047357
1J:268349 Kim HT, Yin W, Jin YJ, Panza P, Gunawan F, Grohmann B, Buettner C, Sokol AM, Preussner J, Guenther S, Kostin S, Ruppert C, Bhagwat AM, Ma X, Graumann J, Looso M, Guenther A, Adelstein RS, Offermanns S, Stainier DYR, Myh10 deficiency leads to defective extracellular matrix remodeling and pulmonary disease. Nat Commun. 2018 Nov 2;9(1):4600
2J:160094 Lemaire R, Farina G, Bayle J, Dimarzio M, Pendergrass SA, Milano A, Whitfield ML, Lafyatis R, Antagonistic effect of the matricellular signaling protein CCN3 on TGF-beta- and Wnt-mediated fibrillinogenesis in systemic sclerosis and Marfan syndrome. J Invest Dermatol. 2010 Jun;130(6):1514-23
4J:306850 Lu T, Lin X, Pan YH, Yang N, Ye S, Zhang Q, Wang C, Zhu R, Zhang T, Wisniewski TM, Cao Z, Ding BS, Dang S, Zhang W, ADAMTS18 Deficiency Leads to Pulmonary Hypoplasia and Bronchial Microfibril Accumulation. iScience. 2020 Aug 20;23(9):101472
1*J:337004 McKnite A, Kim HS, Silva J, Christian JL, Lack of evidence that fibrillin1 regulates bone morphogenetic protein 4 activity in kidney or lung. Dev Dyn. 2023 Jun;252(6):761-769
2J:324217 Mead TJ, Martin DR, Wang LW, Cain SA, Gulec C, Cahill E, Mauch J, Reinhardt D, Lo C, Baldock C, Apte SS, Proteolysis of fibrillin-2 microfibrils is essential for normal skeletal development. Elife. 2022 May 3;11:e71142
1J:329931 Molla MR, Shimizu A, Komeno M, Rahman NIA, Soh JEC, Nguyen LKC, Khan MR, Tesega WW, Chen S, Pang X, Tanaka-Okamoto M, Takashima N, Sato A, Suzuki T, Ogita H, Vascular smooth muscle RhoA counteracts abdominal aortic aneurysm formation by modulating MAP4K4 activity. Commun Biol. 2022 Oct 7;5(1):1071
1J:241460 Nakamichi R, Ito Y, Inui M, Onizuka N, Kayama T, Kataoka K, Suzuki H, Mori M, Inagawa M, Ichinose S, Lotz MK, Sakai D, Masuda K, Ozaki T, Asahara H, Mohawk promotes the maintenance and regeneration of the outer annulus fibrosus of intervertebral discs. Nat Commun. 2016 Aug 16;7:12503
1*J:321553 Neupane S, Berardinelli SJ, Cameron DC, Grady RC, Komatsu DE, Percival CJ, Takeuchi M, Ito A, Liu TW, Nairn AV, Moremen KW, Haltiwanger RS, Holdener BC, O-fucosylation of thrombospondin type 1 repeats is essential for ECM remodeling and signaling during bone development. Matrix Biol. 2022 Feb 12;
2J:295274 Oichi T, Taniguchi Y, Soma K, Oshima Y, Yano F, Mori Y, Chijimatsu R, Kim-Kaneyama JR, Tanaka S, Saito T, Adamts17 is involved in skeletogenesis through modulation of BMP-Smad1/5/8 pathway. Cell Mol Life Sci. 2019 Dec;76(23):4795-4809
2*J:82234 Samuel CS, Sakai LY, Amento EP, Relaxin regulates fibrillin 2, but not fibrillin 1, mRNA and protein expression by human dermal fibroblasts and murine fetal skin. Arch Biochem Biophys. 2003 Mar 1;411(1):47-55
1J:188834 Shen D, Li J, Lepore JJ, Anderson TJ, Sinha S, Lin AY, Cheng L, Cohen ED, Roberts JD Jr, Dedhar S, Parmacek MS, Gerszten RE, Aortic aneurysm generation in mice with targeted deletion of integrin-linked kinase in vascular smooth muscle cells. Circ Res. 2011 Sep 2;109(6):616-28
5J:214897 Shi Y, Tu Y, De Maria A, Mecham RP, Bassnett S, Development, composition, and structural arrangements of the ciliary zonule of the mouse. Invest Ophthalmol Vis Sci. 2013 Apr;54(4):2504-15
3J:62831 Shipley JM, Mecham RP, Maus E, Bonadio J, Rosenbloom J, McCarthy RT, Baumann ML, Frankfater C, Segade F, Shapiro SD, Developmental expression of latent transforming growth factor beta binding protein 2 and its requirement early in mouse development. Mol Cell Biol. 2000 Jul;20(13):4879-87
1J:132011 Sun J, Chen H, Chen C, Whitsett JA, Mishina Y, Bringas P Jr, Ma JC, Warburton D, Shi W, Prenatal lung epithelial cell-specific abrogation of alk3-bone morphogenetic protein signaling causes neonatal respiratory distress by disrupting distal airway formation. Am J Pathol. 2008 Mar;172(3):571-82
1J:278988 Tian C, Johnson KR, TBX1 is required for normal stria vascularis and semicircular canal development. Dev Biol. 2019 Sep 21;
2*J:160041 Tsutsui K, Manabe R, Yamada T, Nakano I, Oguri Y, Keene DR, Sengle G, Sakai LY, Sekiguchi K, ADAMTSL-6 is a novel extracellular matrix protein that binds to fibrillin-1 and promotes fibrillin-1 fibril formation. J Biol Chem. 2010 Feb 12;285(7):4870-82
1J:218158 Turner CJ, Badu-Nkansah K, Crowley D, van der Flier A, Hynes RO, alpha5 and alphav integrins cooperate to regulate vascular smooth muscle and neural crest functions in vivo. Development. 2015 Feb 15;142(4):797-808
3J:334587 Wang L, Lin L, Qi H, Chen J, Grossfeld P, Endothelial Loss of ETS1 Impairs Coronary Vascular Development and Leads to Ventricular Non-Compaction. Circ Res. 2022 Aug 19;131(5):371-387
1*J:275665 Wang LW, Kutz WE, Mead TJ, Beene LC, Singh S, Jenkins MW, Reinhardt DP, Apte SS, Adamts10 inactivation in mice leads to persistence of ocular microfibrils subsequent to reduced fibrillin-2 cleavage. Matrix Biol. 2019 Apr;77:117-128
1J:139011 Wilkinson GA, Schittny JC, Reinhardt DP, Klein R, Role for ephrinB2 in postnatal lung alveolar development and elastic matrix integrity. Dev Dyn. 2008 Aug;237(8):2220-34
2*J:96550 Williams TM, Williams ME, Kuick R, Misek D, McDonagh K, Hanash S, Innis JW, Candidate downstream regulated genes of HOX group 13 transcription factors with and without monomeric DNA binding capability. Dev Biol. 2005 Mar 15;279(2):462-80
6J:321251 Yang N, Zhang Q, Ye S, Lu T, Sun M, Wang L, Wang M, Pan YH, Dang S, Zhang W, Adamts18 Deficiency Causes Spontaneous SMG Fibrogenesis in Adult Mice. J Dent Res. 2022 Feb;101(2):226-234
2J:336178 Yao SQ, Wang M, Liang JJ, Ng TK, Cen LP, Retinal transcriptome of neonatal mice after optic nerve injury. PLoS One. 2023;18(5):e0286344
2J:311873 Ye S, Yang N, Lu T, Wu T, Wang L, Pan YH, Cao X, Yuan X, Wisniewski T, Dang S, Zhang W, Adamts18 modulates the development of the aortic arch and common carotid artery. iScience. 2021 Jun 25;24(6):102672
1J:296772 Yin W, Kim HT, Wang S, Gunawan F, Li R, Buettner C, Grohmann B, Sengle G, Sinner D, Offermanns S, Stainier DYR, Fibrillin-2 is a key mediator of smooth muscle extracellular matrix homeostasis during mouse tracheal tubulogenesis. Eur Respir J. 2019 Mar;53(3)
4*J:22526 Yin W, Smiley E, Germiller J, Sanguineti C, Lawton T, Pereira L, Ramirez F, Bonadio J, Primary structure and developmental expression of Fbn-1, the mouse fibrillin gene. J Biol Chem. 1995 Jan 27;270(4):1798-806
1J:251389 Zakariyah AF, Rajgara RF, Veinot JP, Skerjanc IS, Burgon PG, Congenital heart defect causing mutation in Nkx2.5 displays in vivo functional deficit. J Mol Cell Cardiol. 2017 Apr;105:89-98
3*J:25216 Zhang H, Hu W, Ramirez F, Developmental expression of fibrillin genes suggests heterogeneity of extracellular microfibrils. J Cell Biol. 1995 May;129(4):1165-76

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
Citing These Resources
Funding Information
Warranty Disclaimer, Privacy Notice, Licensing, & Copyright
Send questions and comments to User Support.
last database update
04/16/2024
MGI 6.23
The Jackson Laboratory