Identification |
HMDB Protein ID
| HMDBP13391 |
Secondary Accession Numbers
| None |
Name
| Fibrillin-2 |
Synonyms
|
Not Available
|
Gene Name
| FBN2 |
Protein Type
| Unknown |
Biological Properties |
General Function
| Not Available |
Specific Function
| 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 (PubMed:20855508). |
Pathways
|
Not Available
|
Reactions
| Not Available |
GO Classification
|
Biological Process |
positive regulation of bone mineralization |
limb morphogenesis |
anatomical structure morphogenesis |
camera-type eye development |
bone trabecula formation |
embryonic limb morphogenesis |
sequestering of TGFbeta in extracellular matrix |
positive regulation of osteoblast differentiation |
embryonic eye morphogenesis |
Cellular Component |
microfibril |
extracellular region |
collagen-containing extracellular matrix |
extracellular matrix |
Molecular Function |
extracellular matrix structural constituent |
calcium ion binding |
|
Cellular Location
|
Not Available
|
Gene Properties |
Chromosome Location
| Not Available |
Locus
| Not Available |
SNPs
| Not Available |
Gene Sequence
|
Not Available
|
Protein Properties |
Number of Residues
| 2907 |
Molecular Weight
| 313815.0 |
Theoretical pI
| 4.844 |
Pfam Domain Function
|
|
Signals
|
|
Transmembrane Regions
|
Not Available
|
Protein Sequence
|
Not Available
|
External Links |
GenBank ID Protein
| Not Available |
UniProtKB/Swiss-Prot ID
| Q61555 |
UniProtKB/Swiss-Prot Entry Name
| FBN2_MOUSE |
PDB IDs
|
Not Available |
GenBank Gene ID
| Not Available |
GeneCard ID
| Not Available |
GenAtlas ID
| Not Available |
HGNC ID
| Not Available |
References |
General References
| - Church DM, Goodstadt L, Hillier LW, Zody MC, Goldstein S, She X, Bult CJ, Agarwala R, Cherry JL, DiCuccio M, Hlavina W, Kapustin Y, Meric P, Maglott D, Birtle Z, Marques AC, Graves T, Zhou S, Teague B, Potamousis K, Churas C, Place M, Herschleb J, Runnheim R, Forrest D, Amos-Landgraf J, Schwartz DC, Cheng Z, Lindblad-Toh K, Eichler EE, Ponting CP: Lineage-specific biology revealed by a finished genome assembly of the mouse. PLoS Biol. 2009 May 5;7(5):e1000112. doi: 10.1371/journal.pbio.1000112. Epub 2009 May 26. [PubMed:19468303 ]
- Zhang H, Hu W, Ramirez F: Developmental expression of fibrillin genes suggests heterogeneity of extracellular microfibrils. J Cell Biol. 1995 May;129(4):1165-76. doi: 10.1083/jcb.129.4.1165. [PubMed:7744963 ]
- Li X, Pereira L, Zhang H, Sanguineti C, Ramirez F, Bonadio J, Francke U: Fibrillin genes map to regions of conserved mouse/human synteny on mouse chromosomes 2 and 18. Genomics. 1993 Dec;18(3):667-72. doi: 10.1016/s0888-7543(05)80371-4. [PubMed:8307578 ]
- Arteaga-Solis E, Gayraud B, Lee SY, Shum L, Sakai L, Ramirez F: Regulation of limb patterning by extracellular microfibrils. J Cell Biol. 2001 Jul 23;154(2):275-81. doi: 10.1083/jcb.200105046. [PubMed:11470817 ]
- Carta L, Pereira L, Arteaga-Solis E, Lee-Arteaga SY, Lenart B, Starcher B, Merkel CA, Sukoyan M, Kerkis A, Hazeki N, Keene DR, Sakai LY, Ramirez F: Fibrillins 1 and 2 perform partially overlapping functions during aortic development. J Biol Chem. 2006 Mar 24;281(12):8016-23. doi: 10.1074/jbc.M511599200. Epub 2005 Dec 28. [PubMed:16407178 ]
- Tiedemann K, Boraschi-Diaz I, Rajakumar I, Kaur J, Roughley P, Reinhardt DP, Komarova SV: Fibrillin-1 directly regulates osteoclast formation and function by a dual mechanism. J Cell Sci. 2013 Sep 15;126(Pt 18):4187-94. doi: 10.1242/jcs.127571. [PubMed:24039232 ]
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