Anti-fibrillin 2/FBN2 Antibody Picoband®

SKU: A03408-2

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human

Applications: WB,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: FBN2

Specificity:

Background: Fibrillin is a glycoprotein, which is essential for the formation of elastic fibers found in connective tissue. 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.

Immunogen: E.coli-derived human fibrillin 2/FBN2 recombinant protein (Position: N2740-Y2912).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Babcock, D., Gasner, C., Francke, U., Maslen, C. A single mutation that results in an asp-to-his substitution and partial exon skipping in a family with congenital contractual arachnodactyly. Hum. Genet. 103: 22-28, 1998. 2. Belleh, S., Zhou, G., Wang, M., Der Kaloustian, V. M., Pagon, R. A., Godfrey, M. Two novel fibrillin-2 mutations in congenital contractural arachnodactyly. Am. J. Med. Genet. 92: 7-12, 2000. 3. Lee, B., Godfrey, M., Vitale, E., Hori, H., Mattei, M.-G., Sarfarazi, M., Tsipouras, P., Ramirez, F., Hollister, D. W. Linkage of Marfan syndrome and a phenotypically related disorder to two different fibrillin genes. Nature 352: 330-334, 1991.

Uniprot ID: P35556

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 315 kDa, 250 kDa

Calculated Molecular Weight: 73109 MW

Gene ID: 2201

Protein Name: Fibrillin-2 [Cleaved into: Placensin]

Gene Full Name: fibrillin 2

Synonyms: CCA; DA9; FBN2; fibrillin 2