| Fibrillin-2 is a key mediator of smooth muscle extracellular matrix homeostasis during mouse tracheal tubulogenesis. | Fibrillin-2 is a key mediator of smooth muscle extracellular matrix homeostasis during mouse tracheal tubulogenesis.
Yin W, Kim HT, Wang S, Gunawan F, Li R, Buettner C, Grohmann B, Sengle G, Sinner D, Offermanns S, Stainier DYR. | 10/10/2020 |
| Downstream target genes of Ucma were found in osteoblasts by microarray analysis. 45 genes including Fbn2 were dependent on Ucma overexpression or knockdown. Fbn2 expression was proportional to Ucma expression in osteoblasts. | Unique cartilage matrix-associated protein regulates fibrillin-2 expression and directly interacts with fibrillin-2 protein independent of calcium binding.
Lee YJ, Park SY, Park EK, Kim JE. | 12/28/2019 |
| The analysis of Adamts10-/- mice led to identification of Fbn2 as a novel ADAMTS10 substrate and defined a proteolytic mechanism for clearance of ocular Fbn2 at the end of the juvenile period. | Adamts10 inactivation in mice leads to persistence of ocular microfibrils subsequent to reduced fibrillin-2 cleavage.
Wang LW, Kutz WE, Mead TJ, Beene LC, Singh S, Jenkins MW, Reinhardt DP, Apte SS., Free PMC Article | 08/3/2019 |
| Studies provide evidence for the unique role of fibrillin-2 in regulating BMP-dependent limb patterning and its distinct impact from fbn1 proteins on the commitment and differentiation of marrow mesenchymal stem cells. [review] | Fibrillin microfibrils in bone physiology.
Smaldone S, Ramirez F., Free PMC Article | 12/23/2017 |
| Fibrillin-2 and periostin are target genes in Osterix-mediated osteoblast differentiation. | Induction of fibrillin-2 and periostin expression in Osterix-knockdown MC3T3-E1 cells.
Lee SJ, Lee EH, Park SY, Kim JE. | 01/28/2017 |
| These results suggest that both fibrillin-1 and fibrillin-2 expression is required to form thick oxytalan fibers in periodontal ligament. | Immunohistochemical expression of fibrillin-1 and fibrillin-2 during tooth development.
Kira-Tatsuoka M, Oka K, Tsuruga E, Ozaki M, Sawa Y. | 01/14/2017 |
| Fibrillin-2 plays a role in forelimb muscle differentiation and infiltration of fat into the limb connective tissue space. | Abnormal Activation of BMP Signaling Causes Myopathy in Fbn2 Null Mice.
Sengle G, Carlberg V, Tufa SF, Charbonneau NL, Smaldone S, Carlson EJ, Ramirez F, Keene DR, Sakai LY., Free PMC Article | 05/7/2016 |
| the bronchial epithelial dysplasia accompanying microfibril dysregulation in Adamtsl2(-/-) mice cannot be reversed by TGFbeta neutralization, and thus might be mediated by other mechanisms. | Adamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia--a novel mouse model providing insights into geleophysic dysplasia.
Hubmacher D, Wang LW, Mecham RP, Reinhardt DP, Apte SS., Free PMC Article | 03/5/2016 |
| We propose that the Mp "worse-than-null" eye phenotype plausibly results from a failure in normal trafficking of proteins that are co-expressed with Fbn2 within the ciliary margin | A trans-acting protein effect causes severe eye malformation in the Mp mouse.
Rainger J, Keighren M, Keene DR, Charbonneau NL, Rainger JK, Fisher M, Mella S, Huang JT, Rose L, van't Hof R, Sakai LY, Jackson IJ, Fitzpatrick DR., Free PMC Article | 08/23/2014 |
| Fbn2 has an indispensable role in ocular morphogenesis in mice. The high incidence of iris coloboma in Fbn2-null animals implies a previously unsuspected role in optic fissure closure. | Ocular phenotype of Fbn2-null mice.
Shi Y, Tu Y, Mecham RP, Bassnett S., Free PMC Article | 12/28/2013 |
| fibrillin-2 exerts a greater positive influence on the mechanical properties of bone than fibrillin-1 assemblies | Material and mechanical properties of bones deficient for fibrillin-1 or fibrillin-2 microfibrils.
Arteaga-Solis E, Sui-Arteaga L, Kim M, Schaffler MB, Jepsen KJ, Pleshko N, Ramirez F., Free PMC Article | 08/27/2011 |
| Fibrillin-1 and -2, the structural components of extracellular microfibrils, differentially regulate TGF-beta and bone morphogenetic protein (BMP) bioavailability in bone. | Fibrillin-1 and -2 differentially modulate endogenous TGF-β and BMP bioavailability during bone formation.
Nistala H, Lee-Arteaga S, Smaldone S, Siciliano G, Carta L, Ono RN, Sengle G, Arteaga-Solis E, Levasseur R, Ducy P, Sakai LY, Karsenty G, Ramirez F., Free PMC Article | 10/23/2010 |
| Findings indicate that Fbn2 is involved in integrity of structures required for strength in limb movement. | ENU mutagenesis reveals a novel phenotype of reduced limb strength in mice lacking fibrillin 2.
Miller G, Neilan M, Chia R, Gheryani N, Holt N, Charbit A, Wells S, Tucci V, Lalanne Z, Denny P, Fisher EM, Cheeseman M, Askew GN, Dear TN., Free PMC Article | 10/4/2010 |
| demonstrate a role for fibrillin-2 in bone and soft connective tissue morphological and biochemical processes | Bone and soft connective tissue alterations result from loss of fibrillin-2 expression.
Boregowda R, Paul E, White J, Ritty TM. | 01/21/2010 |
| coordinated expression of the E2F family is critical for the transcriptional regulation of fibrillin-2 during chondrogenesis. | Transcriptional regulation of fibrillin-2 gene by E2F family members in chondrocyte differentiation.
Yanagino T, Yuasa K, Nagahama M, Matsuda Y, Tsuji A. | 01/21/2010 |
| interaction with microfibril-associated glycoprotein-2 | Microfibril-associated glycoprotein-2 interacts with fibrillin-1 and fibrillin-2 suggesting a role for MAGP-2 in elastic fiber assembly.
Penner AS, Rock MJ, Kielty CM, Shipley JM. | 01/21/2010 |
| there are distinct functions for fibrillin-2 in peripheral nerves | Fibrillins can co-assemble in fibrils, but fibrillin fibril composition displays cell-specific differences.
Charbonneau NL, Dzamba BJ, Ono RN, Keene DR, Corson GM, Reinhardt DP, Sakai LY. | 01/21/2010 |
| involvement of fibrillin-2 in the initial assembly of the aortic matrix overlaps in part with fibrillin-1 and continued fibrillin-1 deposition is absolutely required for the maturation and function of the vessel during neonatal life | Fibrillins 1 and 2 perform partially overlapping functions during aortic development.
Carta L, Pereira L, Arteaga-Solis E, Lee-Arteaga SY, Lenart B, Starcher B, Merkel CA, Sukoyan M, Kerkis A, Hazeki N, Keene DR, Sakai LY, Ramirez F., Free PMC Article | 01/21/2010 |
| Relaxin regulates its mRNA and protein expression by human dermal fibroblasts and murine fetal skin | Relaxin regulates fibrillin 2, but not fibrillin 1, mRNA and protein expression by human dermal fibroblasts and murine fetal skin.
Samuel CS, Sakai LY, Amento EP. | 01/21/2010 |