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FBN2

The protein encoded by this gene is a component of connective tissue microfibrils and may be involved in elastic fiber assembly. Mutations in this gene cause congenital contractural arachnodactyly. [provided by RefSeq]
Full Name
fibrillin 2
Research Area
Fibrillin-2:
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.

Placensin:
Hormone secreted by trophoblasts that promotes trophoblast invasiveness (PubMed:32329225).

Has glucogenic activity: is able to increase plasma glucose levels (By similarity).
Biological Process
Anatomical structure morphogenesis Source: GO_Central
Bone trabecula formation Source: BHF-UCL
Camera-type eye development Source: UniProtKB
Embryonic eye morphogenesis Source: UniProtKB
Embryonic limb morphogenesis Source: Ensembl
Positive regulation of bone mineralization Source: BHF-UCL
Positive regulation of osteoblast differentiation Source: BHF-UCL
Sequestering of TGFbeta in extracellular matrix Source: BHF-UCL
Cellular Location
Secreted. Fibrillin-2 and Placensin chains are still linked together during the secretion from cells, but are subsequently separated by furin.
Fibrillin-2: Extracellular matrix
Placensin: Secreted. Secreted by placental cells.
Involvement in disease
Contractural arachnodactyly, congenital (CCA):
An autosomal dominant connective tissue disorder characterized by contractures, arachnodactyly, scoliosis, and crumpled ears.
Macular degeneration, early-onset (EOMD):
An ocular disorder characterized by macular changes resulting in progressive loss of visual acuity.
PTM
Placensin: N-glycosylated.

Anti-FBN2 antibodies

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Target: FBN2
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBXF-0374
Application*: E, IC, IH, IP
Target: FBN2
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: CBXF-3688
Application*: E, WB
Target: FBN2
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: CBXF-0375
Application*: ELISA, WB
Target: FBN2
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBXF-1471
Application*: E, IC, IH, IP
Target: FBN2
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: 1C2
Application*: WB, E
For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
IFImmunofluorescence
IHImmunohistochemistry
IPImmunoprecipitation
WBWestern Blot
EELISA
MMicroarray
CIChromatin Immunoprecipitation
FFlow Cytometry
FNFunction Assay
IDImmunodiffusion
RRadioimmunoassay
TCTissue Culture
GSGel Supershift
NNeutralization
BBlocking
AActivation
IInhibition
DDepletion
ESELISpot
DBDot Blot
MCMass Cytometry/CyTOF
CTCytotoxicity
SStimulation
AGAgonist
APApoptosis
IMImmunomicroscopy
BABioassay
CSCostimulation
EMElectron Microscopy
IEImmunoelectrophoresis
PAPeptide Array
ICImmunocytochemistry
PEPeptide ELISA
MDMeDIP
SHIn situ hybridization
IAEnzyme Immunoassay
SEsandwich ELISA
PLProximity Ligation Assay
ECELISA(Cap)
EDELISA(Det)
BIBioimaging
IOImmunoassay
LFLateral Flow Immunoassay
LALuminex Assay
CImmunohistochemistry-Frozen Sections
PImmunohistologyp-Paraffin Sections
ISIntracellular Staining for Flow Cytometry
MSElectrophoretic Mobility Shift Assay
RIRNA Binding Protein Immunoprecipitation (RIP)
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