NP_034311.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
313,818 Da
NCBI Official Full Name
fibrillin-2
NCBI Official Synonym Full Names
fibrillin 2
NCBI Official Synonym Symbols
sy; sne; Fib-2; BC063774; mKIAA4226 [Similar Products]
NCBI Protein Information
fibrillin-2
UniProt Protein Name
Fibrillin-2
UniProt Synonym Gene Names
UniProt Entry Name
FBN2_MOUSE
UniProt Comments for FBN2
FBN2: Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-2- containing microfibrils regulate the early process of elastic fiber assembly. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. Defects in FBN2 are the cause of distal arthrogryposis type 9 (DA9). A form of distal arthrogryposis, a disease characterized by congenital joint contractures that mainly involve two or more distal parts of the limbs, in the absence of a primary neurological or muscle disease. DA9 is a connective tissue disorder characterized by contractures, arachnodactyly, scoliosis, and crumpled ears. Belongs to the fibrillin family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Secreted, signal peptide; Secreted
Cellular Component: extracellular matrix; proteinaceous extracellular matrix; extracellular region; microfibril
Molecular Function: extracellular matrix structural constituent; calcium ion binding
Biological Process: positive regulation of osteoblast differentiation; positive regulation of bone mineralization; limb morphogenesis; embryonic limb morphogenesis
Research Articles on FBN2
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Products associated with FBN2 sirna
Pathways associated with FBN2 sirna
Diseases associated with FBN2 sirna
Organs/Tissues associated with FBN2 sirna
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