FBN1 Mouse Monoclonal Antibody
- Known as:
- FBN1 Mouse Monoclonal Antibody
- Catalog number:
- BIN-002200-M01
- Product Quantity:
- 0.1mg
- Category:
- -
- Supplier:
- Zyagen
- Gene target:
- FBN1 Mouse Monoclonal Antibody
Related genes to: FBN1 Mouse Monoclonal Antibody
- Gene:
- FBN1 NIH gene
- Name:
- fibrillin 1
- Previous symbol:
- FBN, MFS1, WMS
- Synonyms:
- MASS, OCTD, SGS
- Chromosome:
- 15q21.1
- Locus Type:
- gene with protein product
- Date approved:
- 1987-09-11
- Date modifiied:
- 2019-04-23
Related products to: FBN1 Mouse Monoclonal Antibody
Related articles to: FBN1 Mouse Monoclonal Antibody
- Marfan syndrome (MFS) is a multisystem connective tissue disorder affecting the cardiovascular, ocular, and skeletal systems. We report a case of a 13.5-year-old boy who presented with excessive linear growth. Diagnostic evaluations revealed severe aortic root dilatation, repeatedly positive occult blood in urine, and ultrasonographic findings suggestive of left renal vein entrapment. Genetic testing identified a pathogenic variant in the FBN1 gene. The patient was ultimately diagnosed with Marfan syndrome complicated by left renal vein entrapment syndrome (nutcracker phenomenon). The co-occurrence of severe aortic root dilatation and left renal vein entrapment syndrome in childhood Marfan syndrome is relatively uncommon. This case may provide valuable insights for clinical diagnosis and management. - Source: PubMed
Publication date: 2026/04/14
Qiao XiaoyuChen YanyuSu DanyanShang LifengPang Yusheng - Bladder cancer (BLCA) is a common malignancy of the urinary system, yet the therapeutic relevance of transient receptor potential cation channel subfamily M member 4 (TRPM4) remains unclear. By integrating single-cell and whole-genome transcriptomic data, this study revealed significant transient receptor potential cation channel subfamily M member 4 (TRPM4) overexpression in bladder cancer (BLCA) ( < 0.05), particularly in epithelial cells. Intersection analysis identified 220 candidate genes (7,808 DEGs1, 4,683 DEGs2, and 4,802 key cell module genes). A risk model was constructed comprising six screened prognostic marker genes, namely, protein unc-93 homolog B1 (UNC93B1), family with sequence similarity 193 member B (FAM193B), protein O-glucosyltransferase (POGLUT3), fibrillin-1 (FBN1), microtubule-associated protein 1B (MAP1B), and RUNX family transcription factor 2 (RUNX2). The model demonstrated marked differences among the risk groups. Gene set enrichment analysis revealed significant disparities in key pathways, including the melanoma pathway ( < 0.05). Furthermore, immune infiltration analysis has identified 12 distinct immune cell types, including naive B cells, which showed a < 0.05 distribution. The observed distribution was uneven. In the drug sensitivity analysis, 112 drugs (including WZ3105; < 0.05) showed differential responses, and UNC93B1 showed high positive expression in BLCA tissues (positive cell proportion > 75%). Our studies confirmed that TRPM4 has significant prognostic value and is a potential novel diagnostic and therapeutic target for BLCA. - Source: PubMed
Publication date: 2026/04/13
Zhao QiQin ZitongLiu RunzhangZuo KangweiGuo ChenghaoJing SuoshiLi Weiping - Aortic dissection (AD) is characterized by separation within the medial layers of the aortic wall. Pathogenic variants in the fibrillin-1 gene (FBN1), which cause Marfan syndrome, represent a major genetic cause of AD. In a recently established Fbn1 mouse model, intimomedial tears develop at 3 weeks of age, and 50% of mice die by 5 weeks from aortic rupture. Despite this severe phenotype, the magnitude and expansion of AD lesions, as well as the molecular alterations within the medial layers remain incompletely understood. In this study, we used three-dimensional propagation-based X-ray phase-contrast synchrotron imaging for reconstruction of the ascending aortas, together with single-cell RNA sequencing (scRNA-seq) analysis in Fbn1 mice. Synchrotron imaging revealed 1-2 elastic lamellar breaks evolved into widespread disruptions spanning the entire elastic lamellae, accompanied by localized adventitial thickening. scRNA-seq analysis followed by immunofluorescence staining showed upregulation of fibronectin (Fn1) in Fbn1 smooth muscle cells (SMCs). Consistently, increased FN1 expression was observed in human non-heritable AD samples. Furthermore, enhanced expression of fibronectin receptors and activation of focal adhesion kinase signaling suggested augmented extracellular matrix-SMC interactions during disease progression. These findings indicate that AD progression involves coordinated medial structural failure, adventitial remodeling, and fibronectin-associated SMC dysfunction. - Source: PubMed
Publication date: 2026/04/27
Sheikh Md Al AminKimura KenichiMotoyama EriAsano KeiichiDeleeuw VioletteSips PatrickTokunaga ChihoMatsunaga HirokoKanki SachikoKoizumi ShigekiSugiyama KaoriDe Backer JulieSakai Lynn YTakeyama HarukoHiramatsu YujiOzaki HarukaYanagisawa Hiromi - Asprosin, a fasting-induced adipokine, has been reported to exhibit altered circulating levels in women with polycystic ovary syndrome (PCOS); however, its tissue-specific regulation and the role of its receptor, OLFR734, in PCOS remain poorly understood. In this study, we present the first histological evidence of asprosin and OLFR734 expression in reproductive tissues using dehydroepiandrosterone-induced PCOS mouse models maintained on chow or a high-fat diet (HFD). Metabolic profiling revealed distinct phenotypes, with HFD-fed PCOS mice showing pronounced metabolic disturbances, including increased body weight, glucose intolerance, dyslipidemia, and elevated serum asprosin levels, compared with chow-fed PCOS mice. Asprosin was immunodetected in adipose tissue, ovary, oviduct, and uterus, with increased expression in both PCOS groups. In control ovaries, asprosin was restricted to the theca layer of Graafian follicles. In contrast, PCOS ovaries showed distinct asprosin expression patterns in the theca, granulosa cells, and the antrum of cystic follicles. Asprosin-positive cells were also observed in the oviduct and uterus, with distinct uterine localization in PCOS. OLFR734 showed a similar broad tissue distribution to asprosin, with upregulated expression in both PCOS diet groups. Notably, androgen excess and dietary fat exposure did not produce uniform or additive effects on tissue-level asprosin-OLFR734 expression but instead revealed tissue-specific response patterns under different dietary conditions. Collectively, our findings suggest a potential association of the asprosin-OLFR734 axis with reproductive changes in PCOS, linking metabolic and reproductive dysfunction under diet-induced stress. This work provides a histological and comparative framework, highlighting asprosin and OLFR734 as emerging molecular candidates in PCOS that warrant further investigation for their potential roles in disease pathophysiology. - Source: PubMed
Publication date: 2026/04/25
Khan SanaSyeda SaimaShrivastava Anju - - Source: PubMed
Wang ZiliangLiu YangLu LiliYang LinaYin ShengWang YanQi ZihaoMeng JiaoZang RongyuYang Gong