- Antibody type:Primary
- Antigen name:Fibrillin 2
- Antigen symbol:FBN2
- Clonality:Polyclonal
- Conjugation:FITC (Fluorescein Isothiocyanate)
- Host:Rabbit
- ImmunoChemistry:Yes
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Environmentally Preferable:
- Cross adsorption:No
- Form:liquid
- Antigen synonyms:Fibrillin-2.|congenital contractural arachnodactyly Marfanoid-like|DA9|FBN2|CCA|FBN2_HUMAN|fibrillin 2 congenital contractural arachnodactyly
- Storage buffer:Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
- Storage temperature:Store at 4°C for 12 months
- Concentration:1 μg/μl
- Shipping temperature:4°C
- Purification:Purified by Protein A
- Pk:100 µl
Extracellular glycoproteins fibrillin-1 and -2 are major components of connective tissue microfibrils. Fibrillin-2 containing microfibrils regulate the early process of elastic fiber assembly in tissue. Mutations in the fibrillin-2 gene resulting in impaired assembly of fibrillin-2 may lead to molecular congenital contractural arachnodactyly. Fibrillin-2 constitutes the backbone of microfibrils which insert directly into the lamina densa of basement membranes. Epithelial cells primarily deposit fibrillin into the extracellular matrix in a nonfibrillar form. Mutations in the 8-cysteine motif of Fibrillin-2 alters its binding to microfibril-associated glycoprotein-1 (MAGP-1), which may increase the severity of congenital contractural arachnodactyly.
Recommended Dilutions: IF(IHC-P): 1:50-200
Type: Primary
Antigen: Fibrillin 2
Clonality: Polyclonal
Clone:
Conjugation: FITC (Fluorescein Isothiocyanate)
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat