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Mouse Anti-FLNB Recombinant Antibody (CBXF-0741) (CBMAB-F1721-CQ)

This product is a mouse antibody that recognizes FLNB. The antibody CBXF-0741 can be used for immunoassay techniques such as: IF, IHC-P, WB.
See all FLNB antibodies

Summary

Host Animal
Mouse
Specificity
Human, Rat
Clone
CBXF-0741
Antibody Isotype
IgG2b
Application
IF, IHC-P, WB

Basic Information

Immunogen
Recombinant protein corresponding to a sequence within the N-terminus region of human Filamin B
Specificity
Human, Rat
Antibody Isotype
IgG2b
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Concentration
1.64 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Filamin B
Introduction
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.
Entrez Gene ID
Human2317
Rat306204
UniProt ID
HumanO75369
RatD4A8D5
Alternative Names
Filamin B; Larsen Syndrome 1 (Autosomal Dominant); Actin-Binding-Like Protein; Actin Binding Protein 278; Thyroid Autoantigen; Filamin Homolog 1; Filamin B, Beta; ABP-280 Homolog; Beta-Filamin; Filamin-3; ABP-278; FLN1L; FLN-B; TABP;
Function
Connects cell membrane constituents to the actin cytoskeleton. May promote orthogonal branching of actin filaments and links actin filaments to membrane glycoproteins. Anchors various transmembrane proteins to the actin cytoskeleton. Interaction with FLNA may allow neuroblast migration from the ventricular zone into the cortical plate. Various interactions and localizations of isoforms affect myotube morphology and myogenesis. Isoform 6 accelerates muscle differentiation in vitro.
Biological Process
Actin cytoskeleton organization Source: ProtInc
Cellular response to interferon-gamma Source: Ensembl
Epithelial cell morphogenesis Source: Ensembl
Keratinocyte development Source: Ensembl
Signal transduction Source: ProtInc
Skeletal muscle tissue development Source: Ensembl
Cellular Location
Isoform 1: Cytoskeleton; Stress fiber; Cell cortex; Z line. 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&3: Stress fiber
Isoform 6: Cytoskeleton. Polarized at the periphery of myotubes.
Involvement in disease
Interaction with FLNA may compensate for dysfunctional FLNA homodimer in the periventricular nodular heterotopia (PVNH) disorder.
Atelosteogenesis 1 (AO1):
A lethal chondrodysplasia characterized by distal hypoplasia of the humeri and femurs, hypoplasia of the mid-thoracic spine, occasionally complete lack of ossification of single hand bones, and the finding in cartilage of multiple degenerated chondrocytes which are encapsulated in fibrous tissue.
Atelosteogenesis 3 (AO3):
A short-limb lethal skeletal dysplasia with vertebral abnormalities, disharmonious skeletal maturation, poorly modeled long bones and joint dislocations. Recurrent respiratory insufficiency and/or infections usually result in early death.
Boomerang dysplasia (BOOMD):
A perinatal lethal osteochondrodysplasia characterized by absence or underossification of the limb bones and vertebrae. Patients manifest dwarfism with short, bowed, rigid limbs and characteristic facies. Boomerang dysplasia is distinguished from atelosteogenesis on the basis of a more severe defect in mineralization, with complete absence of ossification in some limb elements and vertebral segments.
Larsen syndrome (LRS):
An osteochondrodysplasia characterized by large-joint dislocations and characteristic craniofacial abnormalities. The cardinal features of the condition are dislocations of the hip, knee and elbow joints, with equinovarus or equinovalgus foot deformities. Spatula-shaped fingers, most marked in the thumb, are also present. Craniofacial anomalies include hypertelorism, prominence of the forehead, a depressed nasal bridge, and a flattened midface. Cleft palate and short stature are often associated features. Spinal anomalies include scoliosis and cervical kyphosis. Hearing loss is a well-recognized complication.
Spondylocarpotarsal synostosis syndrome (SCT):
Disorder characterized by short stature and vertebral, carpal and tarsal fusions.
PTM
ISGylation prevents ability to interact with the upstream activators of the JNK cascade and inhibits IFNA-induced JNK signaling.
Ubiquitination by a SCF-like complex containing ASB2 isoform 1 leads to proteasomal degradation which promotes muscle differentiation.

Wu, H., Wang, Y., Chen, X., Yao, Y., Zhao, W., Fang, L., ... & Xu, C. (2022). Cell-Dependent pathogenic roles of filamin B in different skeletal malformations. Oxidative Medicine and Cellular Longevity, 2022.

Quiggle, A., Charng, W. L., Antunes, L., Nikolov, M., Bledsoe, X., Hecht, J. T., ... & Gurnett, C. A. (2022). Whole exome sequencing in individuals with idiopathic clubfoot reveals a recurrent filamin B (FLNB) deletion. Clinical Orthopaedics and Related Research®, 480(2), 421-430.

Zieba, J., Forlenza, K. N., Heard, K., Martin, J. H., Bosakova, M., Cohn, D. H., ... & Krakow, D. (2022). Intervertebral disc degeneration is rescued by TGFβ/BMP signaling modulation in an ex vivo filamin B mouse model. Bone Research, 10(1), 37.

Liu, C., Tang, W., Zhao, H., Yang, S., Ren, Z., Li, J., ... & Shen, C. (2021). The variants at FLNA and FLNB contribute to the susceptibility of hypertension and stroke with differentially expressed mRNA. The Pharmacogenomics Journal, 21(4), 458-466.

Sankar, S., Younes, S., Ahmad, M. N., Okashah, S. S., Kamaraj, B., Al-Subaie, A. M., & Zayed, H. (2020). Deciphering the role of filamin B calponin-homology domain in causing the Larsen syndrome, boomerang dysplasia, and atelosteogenesis type I spectrum disorders via a computational approach. Molecules, 25(23), 5543.

Ma, H. R., Cao, L., Wang, F., Cheng, C., Jiang, R., Zhou, H., ... & Qian, Z. (2020). Filamin B extensively regulates transcription and alternative splicing, and is associated with apoptosis in HeLa cells. Oncology Reports, 43(5), 1536-1546.

Salian, S., Shukla, A., Shah, H., Bhat, S. N., Bhat, V. R., Nampoothiri, S., ... & Girisha, K. M. (2018). Seven additional families with spondylocarpotarsal synostosis syndrome with novel biallelic deleterious variants in FLNB. Clinical Genetics, 94(1), 159-164.

Czermak, P., Amman, F., Jantsch, M. F., & Cimatti, L. (2018). Organ-wide profiling in mouse reveals high editing levels of Filamin B mRNA in the musculoskeletal system. RNA biology, 15(7), 877-885.

Baudier, J., Jenkins, Z. A., & Robertson, S. P. (2018). The filamin-B–refilin axis–spatiotemporal regulators of the actin-cytoskeleton in development and disease. Journal of Cell Science, 131(8), jcs213959.

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For research use only. Not intended for any clinical use.

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