| In vivo phenotypic vascular dysfunction extends beyond the aorta in a mouse model for fibrillin-1 (Fbn1) mutation. | In vivo phenotypic vascular dysfunction extends beyond the aorta in a mouse model for fibrillin-1 (Fbn1) mutation.
Curry T, Barrameda ME, Thomas TC, Esfandiarei M., Free PMC Article | 03/28/2024 |
| Aqueous humor TGFbeta and fibrillin-1 in Tsk mice reveal clues to POAG pathogenesis. | Aqueous humor TGFβ and fibrillin-1 in Tsk mice reveal clues to POAG pathogenesis.
Tan JC, Ko MK, Woo JI, Lu KL, Kelber JA., Free PMC Article | 02/17/2024 |
| Fibrillin-1 regulates endothelial sprouting during angiogenesis. | Fibrillin-1 regulates endothelial sprouting during angiogenesis.
Alonso F, Dong Y, Li L, Jahjah T, Dupuy JW, Fremaux I, Reinhardt DP, Génot E., Free PMC Article | 06/2/2023 |
| Asprosin aggravates vascular endothelial dysfunction via disturbing mitochondrial dynamics in obesity models. | Asprosin aggravates vascular endothelial dysfunction via disturbing mitochondrial dynamics in obesity models.
Lu Y, Yuan W, Xiong X, Huang Q, Chen S, Yin T, Zhang Y, Wang Z, Zeng G, Huang Q. | 02/26/2023 |
| Exosomal microRNA-133b-3p from bone marrow mesenchymal stem cells inhibits angiogenesis and oxidative stress via FBN1 repression in diabetic retinopathy. | Exosomal microRNA-133b-3p from bone marrow mesenchymal stem cells inhibits angiogenesis and oxidative stress via FBN1 repression in diabetic retinopathy.
Liang G, Qin Z, Luo Y, Yin J, Shi Z, Wei R, Ma W. | 12/24/2022 |
| The critical role of the TB5 domain of fibrillin-1 in endochondral ossification. | The critical role of the TB5 domain of fibrillin-1 in endochondral ossification.
Delhon L, Mougin Z, Jonquet J, Bibimbou A, Dubail J, Bou-Chaaya C, Goudin N, Le Goff W, Boileau C, Cormier-Daire V, Le Goff C. | 11/19/2022 |
| Fibrillin-1 Regulates Arteriole Integrity in the Retina. | Fibrillin-1 Regulates Arteriole Integrity in the Retina.
Alonso F, Li L, Fremaux I, Reinhardt DP, Génot E., Free PMC Article | 11/5/2022 |
| Fibrillin-1 deficiency in the outer perichondrium causes longitudinal bone overgrowth in mice with Marfan syndrome. | Fibrillin-1 deficiency in the outer perichondrium causes longitudinal bone overgrowth in mice with Marfan syndrome.
Sedes L, Wondimu E, Crockett B, Hansen J, Cantalupo A, Asano K, Iyengar R, Rifkin DB, Smaldone S, Ramirez F., Free PMC Article | 10/15/2022 |
| Male Marfan mice are predisposed to high-fat diet-induced obesity, diabetes, and fatty liver. | Male Marfan mice are predisposed to high-fat diet-induced obesity, diabetes, and fatty liver.
Tiedemann K, Muthu ML, Reinhardt DP, Komarova SV. | 08/13/2022 |
| Fibrillin-1 mutant mouse captures defining features of human primary open glaucoma including anomalous aqueous humor TGF beta-2. | Fibrillin-1 mutant mouse captures defining features of human primary open glaucoma including anomalous aqueous humor TGF beta-2.
Ko MK, Woo JI, Gonzalez JM Jr, Kim G, Sakai L, Peti-Peterdi J, Kelber JA, Hong YK, Tan JC., Free PMC Article | 07/2/2022 |
| Fibrillin-1 regulates white adipose tissue development, homeostasis, and function. | Fibrillin-1 regulates white adipose tissue development, homeostasis, and function.
Muthu ML, Tiedemann K, Fradette J, Komarova S, Reinhardt DP. | 06/18/2022 |
| Fibrillin-1-enriched microenvironment drives endothelial injury and vascular rarefaction in chronic kidney disease. | Fibrillin-1-enriched microenvironment drives endothelial injury and vascular rarefaction in chronic kidney disease.
Li L, Liao J, Yuan Q, Hong X, Li J, Peng Y, He M, Zhu H, Zhu M, Hou FF, Fu H, Liu Y., Free PMC Article | 05/7/2022 |
| The Multiple Functions of Fibrillin-1 Microfibrils in Organismal Physiology. | The Multiple Functions of Fibrillin-1 Microfibrils in Organismal Physiology.
Asano K, Cantalupo A, Sedes L, Ramirez F., Free PMC Article | 03/5/2022 |
| Reversion-inducing cysteine-rich protein with Kazal motifs and MT1-MMP promote the formation of robust fibrillin fibers. | Reversion-inducing cysteine-rich protein with Kazal motifs and MT1-MMP promote the formation of robust fibrillin fibers.
Matsuzaki T, Keene DR, Nishimoto E, Noda M., Free PMC Article | 09/18/2021 |
| Asprosin promotes beta-cell apoptosis by inhibiting the autophagy of beta-cell via AMPK-mTOR pathway. | Asprosin promotes β-cell apoptosis by inhibiting the autophagy of β-cell via AMPK-mTOR pathway.
Wang R, Hu W. | 09/4/2021 |
| Fibrillin-1 in the Vasculature: In Vivo Accumulation of eGFP-Tagged Fibrillin-1 in a Knockin Mouse Model. | Fibrillin-1 in the Vasculature: In Vivo Accumulation of eGFP-Tagged Fibrillin-1 in a Knockin Mouse Model.
Charbonneau NL, Manalo EC, Tufa SF, Carlson EJ, Carlberg VM, Keene DR, Sakai LY. | 02/20/2021 |
| asprosin results in impairment of insulin sensitivity in skeletal muscle through PKCdelta-associated endoplasmic reticulum stress/inflammation pathways. | Asprosin attenuates insulin signaling pathway through PKCδ-activated ER stress and inflammation in skeletal muscle.
Jung TW, Kim HC, Kim HU, Park T, Park J, Kim U, Kim MK, Jeong JH. | 09/10/2020 |
| The influence of fibrillin-1 and physical activity upon tendon tissue morphology and mechanical properties in mice. | The influence of fibrillin-1 and physical activity upon tendon tissue morphology and mechanical properties in mice.
Tran PHT, Skrba T, Wondimu E, Galatioto G, Svensson RB, Olesen AT, Mackey AL, Magnusson SP, Ramirez F, Kjaer M., Free PMC Article | 08/29/2020 |
| the present findings show pronounced sex differences in the matrix and function of bones deficient in fibrillin-1 microfibrils. | Assessment of Bones Deficient in Fibrillin-1 Microfibrils Reveals Pronounced Sex Differences.
Altinbas L, Bormann N, Lehmann D, Jeuthe S, Wulsten D, Kornak U, Robinson PN, Wildemann B, Kararigas G., Free PMC Article | 04/11/2020 |
| These results suggest that palmitate-derived asprosin secretion from beta-cells results in their inflammation and dysfunction through a TLR4/JNK-mediated pathway. | Asprosin impairs insulin secretion in response to glucose and viability through TLR4/JNK-mediated inflammation.
Lee T, Yun S, Jeong JH, Jung TW. | 01/18/2020 |
| This model captures key aspects of Marfan-related syndromes, providing insights into the role of fibrillin-1 in eye development and disease. | Targeted deletion of fibrillin-1 in the mouse eye results in ectopia lentis and other ocular phenotypes associated with Marfan syndrome.
Jones W, Rodriguez J, Bassnett S., Free PMC Article | 08/3/2019 |
| Fbn1(C1039G/+) Marfan mouse with extreme aortic dilatation and increased vascular inflammation, which coincided with unilateral renal cystic disease. | Renal cystic disease in the Fbn1(C1039G/+) Marfan mouse is associated with enhanced aortic aneurysm formation.
Hibender S, Wanga S, van der Made I, Vos M, Mulder BJ, Balm R, de Vries CJ, de Waard V. | 03/16/2019 |
| The abundance of elastic fibers was reduced and fragmented in obesity, suggesting that the reduction in elastic fibers is initially caused by increased neprilysin and decreased fibrillin-1 expression, which may inhibit formation and stabilization of elastic fibers, resulting in skin fragility in obesity. | Reduction and fragmentation of elastic fibers in the skin of obese mice is associated with altered mRNA expression levels of fibrillin-1 and neprilysin.
Makihara H, Hidaka M, Sakai Y, Horie Y, Mitsui H, Ohashi K, Goshima Y, Akase T. | 05/5/2018 |
| fibrillin-1 contributes little to lipid storage and metabolic homeostasis, which is in contrast to the obesity and metabolic changes associated with MAGP1 deficiency. | Characterization of metabolic health in mouse models of fibrillin-1 perturbation.
Walji TA, Turecamo SE, DeMarsilis AJ, Sakai LY, Mecham RP, Craft CS., Free PMC Article | 02/24/2018 |
| In young Fbn1(C1039G/+) mice, aortopathy develops in the absence of detectable alterations in smooth muscle cell (SMC) TGF-beta signaling. Loss of physiologic SMC TGF-beta signaling exacerbates MFS-associated aortopathy. Our data support a protective role for SMC TGF-beta signaling during early development of MFS-associated aortopathy. | Aortopathy in a Mouse Model of Marfan Syndrome Is Not Mediated by Altered Transforming Growth Factor β Signaling.
Wei H, Hu JH, Angelov SN, Fox K, Yan J, Enstrom R, Smith A, Dichek DA., Free PMC Article | 01/13/2018 |