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. [provided by RefSeq, Nov 2009].

ABP 278; ABP 280 homolog; ABP-278; ABP-280 homolog; ABP278; Actin binding like protein; Actin binding protein 278; Actin-binding-like protein; AOI; Beta filamin; Beta-filamin; DKFZp686A1668; DKFZp686O033; Fh1; Filamin 1 actin binding protein 280 like; Filamin 3; Filamin B; Filamin B beta actin binding protein 278; Filamin homolog 1; Filamin-3; Filamin-B; FLN B; FLN-B; FLN1L; FLN3; FLNB; FLNB_HUMAN; LRS1; SCT; TABP; TAP; Thyroid autoantigen; Truncated ABP; Truncated actin binding protein; Truncated actin-binding protein.

Human,Mouse,Rat,Dog,Cow,Rabbit,Sheep

WB

WB 1:500-2000

278kD

2317

O75369

https://www.uniprot.org/uniprotkb/O75369

Isoform 1: Cytoplasm, cell cortex. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line. Note=In differentiating myotubes, isoform 1, isoform 2 and isoform 3 are localized diffusely throughout the cytoplasm with regions of enrichment at the longitudinal actin stress fiber. In differentiated tubes, isoform 1 is also detected within the Z-lines.
Isoform 2: Cytoplasm, cytoskeleton. Note=Predominantly localized at actin stress fibers.

Isoform 3: Cytoplasm, cytoskeleton. Note=Predominantly localized at actin stress fibers.

Isoform 6: Cytoplasm, cytoskeleton. Note=Polarized at the periphery of myotubes.

Ubiquitous. Isoform 1 and isoform 2 are expressed in placenta, bone marrow, brain, umbilical vein endothelial cells (HUVEC), retina and skeletal muscle. Isoform 1 is predominantly expressed in prostate, uterus, liver, thyroid, stomach, lymph node, small intestine, spleen, skeletal muscle, kidney, placenta, pancreas, heart, lung, platelets, endothelial cells, megakaryocytic and erythroleukemic cell lines. Isoform 2 is predominantly expressed in spinal cord, platelet and Daudi cells. Also expressed in thyroid adenoma, neurofibrillary tangles (NFT), senile plaques in the hippocampus and cerebral cortex in Alzheimer disease (AD). Isoform 3 and isoform 6 are expressed predominantly in lung, heart, skeletal muscle, testis, spleen, thymus and leukocytes. Isoform 4 and isoform 5 are expressed in heart.

KLH conjugated synthetic peptide derived from human FLNB/Filamin 3

Rabbit

Polyclonal

IgG

affinity purified by Protein A

Liquid

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.