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Anti-Filamin B/FLNB Antibody Picoband® (monoclonal, 11E2D2) Fluoro647 Conjugated

Selected Variant

M02562-1-Fluoro647

Size: 100 µg/Vial

Product Specifications

Background

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.

Synonyms

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

Gene Name

FLNB

Gene ID

2317

UniProt

O75369

Host

Mouse

Reactivity

Human

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

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

Clonality

Monoclonal

Clone

Clone: 11E2D2

Tissue Specificity

Predominantly expressed in skeletal muscle and heart.

Applications

Flow Cytometry

Field of Research

Immunology

Purification

Immunogen affinity purified.

Form

Liquid

Function

Displays temperature-dependent chaperone activity.

References & Citations

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.

Storage Conditions

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

Applications Notes

6

Gene Name Synonym

Heat shock protein family B (small) member 8

Subcellular Location

Nucleus. Cytoplasm.

Protein Name

T-complex protein 1 subunit alpha

Isotype

Mouse IgG1

Contents

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

Price

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