FBN1
fibrillin 1 | MASS, OCTD, SGS, FBN, MFS1, WMS

This gene encodes a member of the fibrillin family of proteins. The encoded preproprotein is proteolytically processed to generate two proteins including the extracellular matrix component fibrillin-1 and the protein hormone asprosin. Fibrillin-1 is an extracellular matrix glycoprotein that serves as a structural component of calcium-binding microfibrils. These microfibrils provide force-bearing structural support in elastic and nonelastic connective tissue throughout the body. Asprosin, secreted by white adipose tissue, has been shown to regulate glucose homeostasis. Mutations in this gene are associated with Marfan syndrome and the related MASS phenotype, as well as ectopia lentis syndrome, Weill-Marchesani syndrome, Shprintzen-Goldberg syndrome and neonatal progeroid syndrome. [provided by RefSeq, Apr 2016]

Biological processes 79 terms
adenylate cyclase-activating G protein-coupled receptor signaling pathway (GO:0007189)angiogenesis (GO:0001525)basement membrane (GO:0005604)calcium ion binding (GO:0005509)calcium ion binding (GO:0005509)camera-type eye development (GO:0043010)camera-type eye development (GO:0043010)cell adhesion mediated by integrin (GO:0033627)cellular response to insulin-like growth factor stimulus (GO:1990314)cellular response to transforming growth factor beta stimulus (GO:0071560)collagen fibril organization (GO:0030199)elastic fiber assembly (GO:0048251)embryonic eye morphogenesis (GO:0048048)embryonic eye morphogenesis (GO:0048048)endoplasmic reticulum lumen (GO:0005788)endothelial cell differentiation (GO:0045446)extracellular matrix (GO:0031012)extracellular matrix (GO:0031012)extracellular matrix (GO:0031012)extracellular matrix (GO:0031012)extracellular matrix (GO:0031012)extracellular matrix constituent conferring elasticity (GO:0030023)extracellular matrix constituent conferring elasticity (GO:0030023)extracellular matrix structural constituent (GO:0005201)extracellular matrix structural constituent (GO:0005201)extracellular matrix structural constituent (GO:0005201)extracellular region (GO:0005576)extracellular region (GO:0005576)extracellular region (GO:0005576)extracellular region (GO:0005576)extracellular region (GO:0005576)gene expression (GO:0010467)gene expression (GO:0010467)glucose homeostasis (GO:0042593)glucose homeostasis (GO:0042593)glucose metabolic process (GO:0006006)glucose metabolic process (GO:0006006)heart development (GO:0007507)heparin binding (GO:0008201)hormone activity (GO:0005179)hormone activity (GO:0005179)hormone activity (GO:0005179)identical protein binding (GO:0042802)immune response (GO:0006955)inflammatory response (GO:0006954)integrin binding (GO:0005178)intracellular protein localization (GO:0008104)kidney development (GO:0001822)lung alveolus development (GO:0048286)lung development (GO:0030324)metanephros development (GO:0001656)microfibril (GO:0001527)microfibril (GO:0001527)negative regulation of BMP signaling pathway (GO:0030514)negative regulation of BMP signaling pathway (GO:0030514)negative regulation of osteoclast development (GO:2001205)negative regulation of osteoclast development (GO:2001205)negative regulation of osteoclast differentiation (GO:0045671)negative regulation of osteoclast differentiation (GO:0045671)negative regulation of transforming growth factor beta receptor signaling pathway (GO:0030512)negative regulation of transforming growth factor beta receptor signaling pathway (GO:0030512)positive regulation of appetite (GO:0032100)post-embryonic eye morphogenesis (GO:0048050)protein binding (GO:0005515)protein-containing complex binding (GO:0044877)regulation of plasma lipoprotein particle levels (GO:0097006)response to ATP (GO:0033198)response to acetylcholine (GO:1905144)response to mechanical stimulus (GO:0009612)response to nitric oxide (GO:0071731)response to oxidative stress (GO:0006979)response to peptide (GO:1901652)response to xenobiotic stimulus (GO:0009410)signal transduction (GO:0007165)signaling receptor binding (GO:0005102)skeletal system development (GO:0001501)skin development (GO:0043588)triglyceride homeostasis (GO:0070328)vasodilation (GO:0042311)
Expression (TPM)
FBN1 — as a Regulated Gene

TFs regulating FBN1 0 TFs

Transcription factors with Perturb-seq knockdown data for FBN1. The Binding column indicates whether any binding evidence exists for this TF–gene pair (ChIP-seq or motif footprint peaks). The Mean coef is the average Perturb-seq regression coefficient across active gRNAs (positive = FBN1 upregulated upon KD; negative = downregulated). The Outlier column indicates whether this gene is in the top or bottom 5% of all TF knockdown effects.

Data: Effect:
TF Mean coef Binding Outlier TF→Gene link

Elements linked to FBN1

Open chromatin peaks (ATAC-seq) in the genomic neighbourhood of FBN1, linked by TSS proximity or chromatin conformation (Multiome / HiCAR). Each element overlaps at least one TF ChIP-seq binding site — the TFs column shows how many distinct TFs bind that element.

Accessibility Element Dist. to TSS Link type TFs
chr15:48,644,398–48,646,594 182 bp At TSS Multiome 507
chr15:48,710,844–48,711,587 65.4 kb Distal (>10kb) Multiome 181
chr15:48,810,417–48,812,023 165.5 kb Distal (>10kb) Multiome 720
chr15:48,877,739–48,878,642 232.4 kb Distal (>10kb) Multiome 662

Genome Browser

Genomic view of the FBN1 locus showing ATAC-seq accessibility and RNA-seq expression across the ESC → DE time course, together with TF binding peaks and element-to-TSS loop connections.

chr15:48,634,398 – 48,888,642
Proximal 1 kb Distal 10 kb Multiome HiCAR ATAC-seq RNA-seq