FLNA native protein, Filamin native protein

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Filamin native protein

Catalog Number: 11-663-46181

Buy FLNA native protein -

Size:

Trial Size Available.

Related Product Names:

- FLNA native protein; FLNA; FLN; FLN1; Filamin
- Filamin; Alpha-filamin; Filamin-1; Endothelial actin-binding protein; Actin-binding protein 280; ABP-280; Non-muscle filamin; filamin

- Gene Information - Information in yellow represents specific gene information and does not necessarily represent specific product details. For more information please contact sales@genwaybio.com.

Gene Name: FLNA	Gene Name Synonym: FLN; FLN1
Gi #: N/A	NCBI Acc #: NP_001104026.1	Swiss Prot Acc #: P21333
Length (aa): N/A	Mol. Weight (Da): 280739	Chrom Location: N/A

Physical Appearance: Sterile Filtered White lyophilized (freeze-dried) powder.

Solubility: It is recommended to reconstitute the lyophilized Filamin in sterile 18M-cm H2O not less than 100ug/ml, which can then be further diluted to other aqueous solutions.

Useful Applications: Protein standard in 1D and 2D SDS gelelectrophoresis
Immunoassays Immunization

Source/Host: Chkn (Gizzard)

Purity/Purification: Greater than 90.0% as determined by:
(a) Analysis by RP-HPLC.
(b) Analysis by SDS-PAGE.

Format: The protein was lyophilized from a 1mg/ml solution containing 20 mM Tris / acetate buffer pH 7.6, 0.1mM EDTA, 2 mM DTT and 20 mM NaCl. Each mg Filamin contains 550mg urea.

Stability: Lyophilized Filamin although stable at room temperature for 3 weeks, should be stored desiccated below -18 C. Upon reconstitution Filamin should be stored at 4 C between 2-7 days and for future use below -18 C.
For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Please prevent freeze-thaw cycles.

Shipping: Products may be shipped on ice pack or dry ice.

TESTING: (secondary reagents and protocols )

Not Available

FLNA NATIVE PROTEIN TARGET DESCRIPTION:

Synonym Names for FLNA native protein: FLNA; FLN; FLN1; Filamin; Alpha-filamin; Filamin-1; Endothelial actin-binding protein; Actin-binding protein 280; ABP-280; Non-muscle filamin; filamin

Ultra Pure Filamin having a Molecular mass of 250 kDa.

Function: Promotes orthogonal branching of actin filaments and links actin filaments to membrane glycoproteins. Anchors various transmembrane proteins to the actin cytoskeleton and serves as a scaffold for a wide range of cytoplasmic signaling proteins. Interaction with FLNA may allow neuroblast migration from the ventricular zone into the cortical plate. Tethers cell surface-localized furin, modulates its rate of internalization and directs its intracellular trafficking (By similarity).

Subunit: Interacts with PDLIM2 (By similarity). Homodimer. Interacts with FCGR1A, FLNB, FURIN, HSPB7, INPPL1, KCND2, MYOT, MYOZ1, ARHGAP24, PSEN1 and PSEN2. Interacts also with various other binding partners in addition to filamentous actin.

Subcellular Location: Cytoplasm, cell cortex. Cytoplasm, cytoskeleton.

Tissue Specificity: Ubiquitous.

Domain: Comprised of a NH2-terminal actin-binding domain, 24 internally homologous repeats and two hinge regions. Repeat 24 and the second hinge domain are important for dimer formation.

Ptm: Phosphorylated upon DNA damage, probably by ATM or ATR (By similarity). Phosphorylation extent changes in response to cell activation.

Ptm: The N-terminus is blocked.

Disease: Defects in FLNA are the cause of periventricular nodular heterotopia type 1 (PVNH1) [MIM:300049]; also called nodular heterotopia, bilateral periventricular (NHBP or BPNH). PVNH is a developmental disorder characterized by the presence of periventricular nodules of cerebral gray matter, resulting from a failure of neurons to migrate normally from the lateral ventricular proliferative zone where they are formed, to the cerebral cortex. PVNH1 is an X-linked dominant form. Heterozygous females have normal intelligence but suffer from seizures and various manifestations outside the central nervous system, especially related to the vascular system. Hemizygous affected males die in the prenatal or perinatal period.

Disease: Defects in FLNA are the cause of periventricular nodular heterotopia type 4 (PVNH4) [MIM:300537]; also known as periventricular heterotopia Ehlers-Danlos variant. PVNH4 is characterized by nodular brain heterotopia, joint hypermobility and development of aortic dilatation in early adulthood.

Disease: Defects in FLNA are the cause of otopalatodigital syndrome type 1 (OPD1) [MIM:311300]. OPD1 is an X-linked dominant multiple congenital anomalies disease mainly characterized by a generalized skeletal dysplasia, mild mental retardation, hearing loss, cleft palate, and typical facial anomalies. OPD1 belongs to a group of X-linked skeletal dysplasias known as oto-palato-digital syndrome spectrum disorders that also include OPD2, Melnick-Needles syndrome (MNS), and frontometaphyseal dysplasia (FMD). Remodeling of the cytoskeleton is central to the modulation of cell shape and migration. FLNA is a widely expressed protein that regulates re-organization of the actin cytoskeleton by interacting with integrins, transmembrane receptor complexes and second messengers. Males with OPD1 have cleft palate, malformations of the ossicles causing deafness and milder bone and limb defects than those associated with OPD2. Obligate female carriers of mutations causing both OPD1 and OPD2 have variable (often milder) expression of a similar phenotypic spectrum.

Disease: Defects in FLNA are the cause of otopalatodigital syndrome type 2 (OPD2) [MIM:304120]; also known as cranioorodigital syndrome. OPD2 is a congenital bone disorder that is characterized by abnormally modeled, bowed bones, small or absent first digits and, more variably, cleft palate, posterior fossa brain anomalies, omphalocele and cardiac defects.

Disease: Defects in FLNA are the cause of frontometaphyseal dysplasia (FMD) [MIM:305620]. FMD is a congenital bone disease characterized by supraorbital hyperostosis, deafness and digital anomalies.

Disease: Defects in FLNA are the cause of Melnick-Needles syndrome (MNS) [MIM:309350]. MNS is a severe congenital bone disorder characterized by typical facies (exophthalmos, full cheeks, micrognathia and malalignment of teeth), flaring of the metaphyses of long bones, s-like curvature of bones of legs, irregular constrictions in the ribs, and sclerosis of base of skull.

Disease: Defects in FLNA are associated with cerebrofrontofacial syndrome [MIM:608578]. This syndrome consists of a phenotype of male PVNH, with relatively normal development, no epilepsy or other neurological abnormality, severe constipation, and facial dysmorphism and without a discernible skeletal phenotype.

Disease: Defects in FLNA are the cause of X-linked congenital idiopathic intestinal pseudoobstruction (CIIPX) [MIM:300048]. CIIPX is characterized by a severe abnormality of gastrointestinal motility due to primary qualitative defects of enteric ganglia and nerve fibers. Affected individuals manifest recurrent signs of intestinal obstruction in the absence of any mechanical lesion.

Similarity: Belongs to the filamin family.

Similarity: Contains 1 actin-binding domain.

Similarity: Contains 2 CH (calponin-homology) domains.

Similarity: Contains 24 filamin repeats.

OMIM: 300017; gene. [NCBI / EBI]
300048; phenotype. [NCBI / EBI]
300049; phenotype. [NCBI / EBI]
300537; phenotype. [NCBI / EBI]
304120; phenotype. [NCBI / EBI]
305620; phenotype. [NCBI / EBI]
309350; phenotype. [NCBI / EBI]
311300; phenotype. [NCBI / EBI]
608578; phenotype. [NCBI / EBI]

Products similar to FLNA native protein:

IgG

FLNB, IgG

FILAMIN, IgG

Filamin 90kDa, IgG

TRAFD1 (TRAF-type zinc finger domain containing 1), IgG

FILAMIN, IgG

PRODUCT INTERACTIONS:

Filamin interacts with c-Abl phospho Y245, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with c-Abl (Phospho-Tyr412), IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with c-Abl (Ab-412), IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with c-Abl (Phospho-Tyr412) Antibody Blocking Peptide, Peptide (GenWay Catalog #: 11-663-46181).

Filamin interacts with Herpes Type 6 p150, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with c-Abl (ready-to-use), IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with c-Abl, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with c-Abl [19-110], IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with c-Abl [ABL-148], IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with c-Abl phospho Y412, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with c-Abl, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with v-crk sarcoma virus CT10 oncogene homolog (avian) (CRK), transcript variant II, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with Crk (p38), IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with Crk / p38 Phospho (pY221), IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with FYN, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with FYN [FYN-01], IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with Fyn-p59 Human, Protein (GenWay Catalog #: 11-663-46181).

Filamin interacts with FYN, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with Fyn (p59Fyn), IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with Growth factor receptor-bound protein 2, Protein (GenWay Catalog #: 11-663-46181).

Filamin interacts with Grb2, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with GRB2 [R11], IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with GRB2 [R13], IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with Grb2, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with Grb2, C-Term, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with GRB2 (N-term), IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with Recombinant Human Growth Factor Receptor-Bound Protein 2, Protein (GenWay Catalog #: 11-663-46181).

Filamin interacts with p85a Bovine, Protein (GenWay Catalog #: 11-663-46181).

Filamin interacts with p85 alpha, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with PI 3 Kinase p85 alpha [U13], IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with PI 3 Kinase p85, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with PI-3 KINASE, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with PI3-kinase p85 subunit alpha (C-term), IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with PLC-gamma1 (Phospho-Tyr783), IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with PLC-gamma1 (Ab-783), IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with PLC 1 (Phospho-Tyr783) Antibody Blocking Peptide, Peptide (GenWay Catalog #: 11-663-46181).

Filamin interacts with PLC 2(Phospho-Tyr753) Antibody Blocking Peptide, Peptide (GenWay Catalog #: 11-663-46181).

Filamin interacts with Phospholipase C1, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with Phospholipase C gamma 1 phospho Y783, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with PLC I (pY783) Phospho, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with PLC y-1, IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with Phospho PLC gamma I (pY1245), IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with PLC I (C-term), IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with PLC Gamma I Phospho (pY783), IgG (GenWay Catalog #: 11-663-46181).

Filamin interacts with PLC I Phospho (pY771), IgG (GenWay Catalog #: 11-663-46181).

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Precautions: FLNA native protein is for in vitro research use only. Not for use in diagnostics or therapeutic procedures.

Important Notes: During shipment, small volumes of FLNA native protein vial. For products with volumes of 200 µL or less, we recommend gently tapping the vial on a hard surface or briefly centrifuging the vial in a tabletop centrifuge to dislodge any liquid in the container’s cap. Actual concentration, volume and quantity will be printed on the vial's label. Please refer to the vials label for this information.

Copyright: This GenWay TDS is copyrighted. This datasheet is produced based partially on data from Swiss-Prot/TrEMBL and NCBI. To better serve our clients with everything we know about FLNA native protein, all related information, articles, resources about FLNA native protein are being stored on our online database. Let us know if you have questions regarding this product.

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