FBN1 antibody - N-terminal region (ARP37969_P050)
- Known as:
- FBN1 (anti-) - N-terminal region (ARP37969_P050)
- Catalog number:
- arp37969_p050
- Product Quantity:
- USD
- Category:
- -
- Supplier:
- Aviva Systems Biology
- Gene target:
- FBN1 antibody - N-terminal region (ARP37969_P050)
Related genes to: FBN1 antibody - N-terminal region (ARP37969_P050)
- 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 antibody - N-terminal region (ARP37969_P050)
Related articles to: FBN1 antibody - N-terminal region (ARP37969_P050)
- Spontaneous coronary artery dissection (SCAD) is characterized by a separation of the coronary artery wall, causing myocardial infarction and sudden death. This study is one of the first to clarify the impact of pathological genetic variants in candidate SCAD-related genes on the histopathological and morphological properties of human SCAD lesions. - Source: PubMed
Publication date: 2026/09/01
Tanaka TakamasaKawakami RikaGaynor Brady JWilliams DesireeAugenstreich JacquesSakamoto AtsushiJinnouchi HiroyukiKawai KenjiKonishi TakaoShiraki TatsuyaSekimoto TeruoNakayama TakafumiFujiyoshi KazuhiroHamana TomoyoAdachi YusukeDiaz Keisha MedinaHong Charles CGrogan AlyssaMitchell Braxton DVirmani RenuFinn Aloke V - Fibrillins constitute a class of extracellular matrix proteins, and the C-terminal peptide of vertebrate fibrillin members has been identified as a hormone that regulates glucose metabolism. Their evolutionary history has attracted increasing attention recently as the hormonal roles seemingly originated in invertebrates. However, the evolution of fibrillin homologs in invertebrates such as insects, remains poorly understood. In this study, 373 annotated fibrillin sequences (including fibrillin-1, fibrillin-2, and fibrillin-3) from 181 species, 126 genera, 65 families, and 12 insect orders were retrieved and analyzed. Multidimensional analysis of phylogenesis, sequence features, conserved motifs, functional domains, and three-dimensional structures was performed on these sequences to elucidate their evolutionary relationships. Phylogenetic analysis categorized all annotated insects fibrillin sequences into three distinct clades. Among them, only the fibrillin-2 annotated members exhibit clear homology to the vertebrate fibrillin family, having diverged early from a putative ancestral protein together with the other two fibrillin annotated groups. Analysis of cysteine ratio, motif, domain composition, and furin cleavage site also revealed that only insect fibrillin-2 annotated homologs share highly similar sequence and structural features with vertebrate fibrillins. By contrast, the other two insect fibrillin annotated members are more divergent from the canonical fibrillin. Combined analyses of sequence conservation, phylogeny, domain analysis, and three-dimensional structural comparisons demonstrates that insect annotated fibrillin-1 proteins are more closely related to vertebrate fibulin than to canonical fibrillins, whereas insect annotated fibrillin-3 proteins may represent an uncharacterized insect-specific protein family associated with cell adhesion. These two groups are closely related to proteins of fibrillin family and have been misannotated in public database. - Source: PubMed
Publication date: 2026/08/24
Chen JianiZhou FengSun XinyuLi ZhuanxiaSun ShuningZhang YuyingWan Lixia - Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder. The clinical manifestations are pleiotropic, predominantly affecting the cardiovascular system (atrioventricular valve prolapse and regurgitation and aortic complications), the skeletal system (disproportionate elongation of long bones), and the ocular system (ectopia lentis and myopia). Pathogenic variants in the fibrillin-1 gene (FBN1) are the primary genetic cause of MFS. - Source: PubMed
Publication date: 2026/08/28
Zhao XingYuan LameiSun YanXia HongHu PengzhiXu HongboGong LinaDeng Hao - To explore the correlation between clinical manifestations and variants of fibrillin-1 (FBN1) gene in eight patients with Marfan syndrome (MFS). - Source: PubMed
Wu RenhuaSun LeiLiu BaozhuFu XiaoLi XiaoyanWang Yueli - Marfan syndrome is a connective tissue disorder affecting the cardiovascular, skeletal, and ocular systems. Here, we generated and characterized induced pluripotent stem cell (iPSC) lines derived from two Marfan syndrome patients with mutations in the FBN1 gene (c.3333C > A and c.8854_8562delinsTATCAC). Both lines exhibited typical iPSC morphology, normal karyotype, undifferentiated states, and trilineage differentiation capacity. These iPSCs serve to enable investigation into the mechanisms underlying Marfan syndrome for therapeutic discovery. - Source: PubMed
Publication date: 2026/08/22
Mui Byron W HChorsi Mey-SamYan Christopher DChao Jade TWu Joseph C