Anti-Filamine B/FLNB Antibody Picoband® (monoklonale, 11E2D2) fluoro594

Filamin B, beta (FLNB), also known as Filamin B, beta (truncated actin binding protein 278 homolog), is a cytoplasmic protein which in humans is encoded by the FLNB gene. This gene encodes a member of the filamin family. The encoded protein interacts with glycoprotein Ib alpha as part of the process to repair vascular injuries. The platelet glycoprotein Ib complex includes glycoprotein Ib alpha, and it binds the actin cytoskeleton. Mutations in this gene have been found in several conditions: atelosteogenesis type 1 and type 3; boomerang dysplasia; autosomal dominant Larsen syndrome; and spondylocarpotarsal synostosis syndrome. Multiple alternatively spliced transcript variants that encode different protein isoforms have been described for this gene.

CMT2L; CRYAC; DHMN 2; DHMN2; E2IG1; H11; HMN 2; HMN2; HMN2A; HSB8; HSPB 8; HspB8

FLNB

2317

O75369

Human

No cross-reactivity with other proteins.

E.coli-derived human Filamin B/FLNB recombinant protein (Position: Q397-D701) .

Clone: 11E2D2

Predominantly expressed in skeletal muscle and heart.

Flow Cytometry

Immunology

Immunogen affinity purified.

Liquid

Displays temperature-dependent chaperone activity.

1. Bicknell, L. S., Farrington-Rock, C., Shafeghati, Y., Rump, P., Alanay, Y., Alembik, Y., Al-Madani, N., Firth, H., Karimi-Nejad, M. H., Kim, C. A., Leask, K., Maisenbacher, M., and 14 others. A molecular and clinical study of Larsen syndrome caused by mutations in FLNB. J. Med. Genet. 44: 89-98, 2007. 2. Bicknell, L. S., Morgan, T., Bonafe, L., Wessels, M. W., Bialer, M. G., Willems, P. J., Cohn, D. H., Krakow, D., Robertson, S. P. Mutations in FLNB cause boomerang dysplasia. J. Med. Genet. 42: e43, 2005. Note: Electronic Article. 3. Biesecker, L. G. Phenotype matters. Nature Genet. 36: 323-324, 2004.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

6

Heat shock protein family B (small) member 8

Nucleus. Cytoplasm.

T-complex protein 1 subunit alpha

Mouse IgG1

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.