fibrillin
Sign in to saveAlso known as Fibrillin 1, unique N-terminal domain, protein family, IPR040872, FBN, fibrillins
Fibrillin is a glycoprotein, which is essential for the formation of elastic fibers found in connective tissue. Fibrillin is secreted into the extracellular matrix by fibroblasts and becomes incorporated into the insoluble microfibrils, which appear to provide a scaffold for deposition of elastin.
Key facts
- Protein.OMIM
- 608529
- Protein.Name
- fibrillin 3
- Protein.caption
- Crystallographic structure of the cbEGF9-hybrid2-cbEGF10 region of human fibrillin 1.
- Protein.image
- 2W86 (Fibrillin).png
- Protein.HGNCid
- 18794
- Protein.Symbol
- FBN3
- Protein.AltSymbols
- CCA
- Protein.EntrezGene
- 84467
- Protein.RefSeq
- NM_032447
- Protein.UniProt
- P35556
- Protein.PDB
- 2W86
- Protein.Chromosome
- 5
- Protein.Arm
- q
- Protein.Band
- 23
- Protein.LocusSupplementaryData
- -q31
via Wikipedia infobox
Wikidata facts
- Image
- 2W86 (Fibrillin).png
Show 1 more fact
- Commons category
- Fibrillin
via Wikidata · CC0
~2 min read
Article
9 sectionsContents
- Clinical aspects
- Types
- Fibrillin-1
- Structure
- Fibrillin-2
- Fibrillin-3
- Fibrillin-4
- References
- External links
Fibrillin is a glycoprotein, which is essential for the formation of elastic fibers found in connective tissue. Fibrillin is secreted into the extracellular matrix by fibroblasts and becomes incorporated into the insoluble microfibrils, which appear to provide a scaffold for deposition of elastin.
==Clinical aspects== Marfan syndrome is a genetic disorder of the connective tissue caused by defected FBN1 gene. Mutations in FBN1 and FBN2 are also sometimes associated with adolescent idiopathic scoliosis.