Ms X Hu Filamin A

Species Reactivity	Key Applications	Host	Format	Antibody Type
Ch, Gp, H, M, R, Rb	IF, IHC, IH(P), IP, WB	M	Ascites	Monoclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

Ch, Gp, H, M, R, Rb

IF, IHC, IH(P), IP, WB

Ascites

Monoclonal Antibody

Description
Catalogue Number	MAB1678
Brand Family	Chemicon®
Trade Name	Chemicon
Description	Anti-Filamin A Antibody, clone PM6/317
Alternate Names	Alpha-Filamin Filamin I Endothelial Actin-binding Protein ABP-280 Nonmuscle Filamin
Background Information	Filamin is a structural protein that forms flexible cross-links between two actin filaments. Filamin is a homodimer of polypeptide chains each joined to the other at one end with an actin binding site ath the other. It is present in smooth muscle, fibroblasts, platelets and lymphocytes.

Description

Catalogue Number

MAB1678

Brand Family

Chemicon®

Trade Name

Chemicon

Description

Anti-Filamin A Antibody, clone PM6/317

Alternate Names

Alpha-Filamin
Filamin I
Endothelial Actin-binding Protein
ABP-280
Nonmuscle Filamin

Background Information

Filamin is a structural protein that forms flexible cross-links between two actin filaments. Filamin is a homodimer of polypeptide chains each joined to the other at one end with an actin binding site ath the other. It is present in smooth muscle, fibroblasts, platelets and lymphocytes.

Product Information
Format	Ascites
Control	Positive control tisse: skin, jurkat cell lysate.
Presentation	Mouse monoclonal ascites IgG1 in buffer containing 0.1% sodium azide.

Product Information

Format

Ascites

Control

Positive control tisse: skin, jurkat cell lysate.

Presentation

Mouse monoclonal ascites IgG1 in buffer containing 0.1% sodium azide.

Applications
Application	This Anti-Filamin A Antibody, clone PM6/317 is validated for use in IF, IH, IH(P), IP, WB for the detection of Filamin A.
Key Applications	Immunofluorescence Immunohistochemistry Immunohistochemistry (Paraffin) Immunoprecipitation Western Blotting
Application Notes	Immunoblotting: 1:1000-1:4000. Because of the large size of the unprocessed forms of filamin, 4-7% PAGE gels and proteinase inhibitors are recommended. Immunofluorescence: 1:50-1:200 dilution from a previous lot was used. Suitable for staining both frozen and paraffin embedded tissues (at lower dilutions). Microwave-citrate buffer antigen retrieval method recommended for paraffin sections. Immunoprecipitation: A previous lot was used on immunoprecipitation. Suggested lysis buffer is PBS with 0.5% triton X-100 with proteinase inhibitors (note for full length filamin include calpain inhibitors). 5 microliters of antibody for every 300-500 μL of cell lysate (200-500 μg/mL total protein is suggested. Incubation is 1 hour RT or overnight 4C; Protein A/G agarose beads or rabbit anti-mouse secondary capture antibody is recommended for best recovery. 4-8% acrylamide gels are recommended for full length filamin or the 190 kDa fragement visualization. Optimal working dilutions must be determined by end user.

Applications

Application

This Anti-Filamin A Antibody, clone PM6/317 is validated for use in IF, IH, IH(P), IP, WB for the detection of Filamin A.

Key Applications

Immunofluorescence
Immunohistochemistry
Immunohistochemistry (Paraffin)
Immunoprecipitation
Western Blotting

Application Notes

Immunoblotting:
1:1000-1:4000. Because of the large size of the unprocessed forms of filamin, 4-7% PAGE gels and proteinase inhibitors are recommended.

Immunofluorescence:
1:50-1:200 dilution from a previous lot was used. Suitable for staining both frozen and paraffin embedded tissues (at lower dilutions). Microwave-citrate buffer antigen retrieval method recommended for paraffin sections.

Immunoprecipitation:
A previous lot was used on immunoprecipitation. Suggested lysis buffer is PBS with 0.5% triton X-100 with proteinase inhibitors (note for full length filamin include calpain inhibitors). 5 microliters of antibody for every 300-500 μL of cell lysate (200-500 μg/mL total protein is suggested. Incubation is 1 hour RT or overnight 4C; Protein A/G agarose beads or rabbit anti-mouse secondary capture antibody is recommended for best recovery. 4-8% acrylamide gels are recommended for full length filamin or the 190 kDa fragement visualization.

Optimal working dilutions must be determined by end user.

Biological Information
Immunogen	Human platelet protein.
Clone	PM6/317
Concentration	Please refer to the Certificate of Analysis for the lot-specific concentration.
Host	Mouse
Specificity	Recognizes unprocessed, full length Human filamin (actin-binding protein; 270-280 kDa) as well as the 190 kDa N-terminal calpain cleavage fragment of filamin (Aakhus, 1992). Following induction of apoptosis in U937 cells, the antibody recognizes 170, 150, and 120 kDa N-terminal cleavage fragments of the full-length form presumably resulting from cleavage by activated caspase-3 (Umeda, 2001).
Isotype	IgG1
Species Reactivity	Chicken Guinea Pig Human Mouse Rat Rabbit
Species Reactivity Note	Human, mouse, and rat. Expected to cross-react with chicken, guinea pig and rabbit.
Antibody Type	Monoclonal Antibody
Entrez Gene Number	NM_001110556.1
Entrez Gene Summary	Actin-binding protein, or filamin, is a 280-kD protein that crosslinks actin filaments into orthogonal networks in cortical cytoplasm and participates in the anchoring of membrane proteins for the actin cytoskeleton. Remodeling of the cytoskeleton is central to the modulation of cell shape and migration. Filamin A, encoded by the FLNA gene, is a widely expressed protein that regulates reorganization of the actin cytoskeleton by interacting with integrins, transmembrane receptor complexes, and second messengers.[supplied by OMIM]
Gene Symbol	FLNA Alpha-filamin OPD2 Filamin-A MNS FLN FMD OPD1 FLN1 NHBP ABP-280 ABPX OPD Filamin-1 DKFZp434P031
Purification Method	Unpurified
UniProt Number	P21333
UniProt Summary	FUNCTION: SwissProt: P21333 # 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). SIZE: 2647 amino acids; 280739 Da SUBUNIT: Interacts with PDLIM2 (By similarity). Homodimer. Interacts with 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. TISSUE SPECIFICITY: Ubiquitous. DOMAIN: SwissProt: P21333 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. & The N-terminus is blocked. DISEASE: SwissProt: P21333 # Defects in FLNA are the cause of periventricular nodular heterotopia 1 (PVNH1) [MIM:300049]; also called nodular heterotopia, bilateral periventricular (NHBP or BPNH). PVNH1 is an X-linked developmental dominant disorder in which many neurons fail to migrate into the cerebral cortex. They remain as nodules lining the ventricular surface. In heterozygous females these neurons presumably represent those cells that, after X-chromosome inactivation, contain the active X chromosome with the filamin mutation. Most hemizygous affected males die early during embryogenesis, whereas heterozygous females have normal intelligence but suffer from seizures and various manifestations outside the central nervous system, especially related to the vascular system. This implies that essential embryonic cell migration can only occur in FLNA-expressing cells. & Defects in FLNA are the cause of periventricular nodular heterotopia 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. & 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. & 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. & 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. & 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. & 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. & Defects in FLNA are a cause of X-linked congenital idiopathic intestinal pseudoobstruction (CIIPX) [MIM:300048]. CIIPX is caused by severe abnormality of gastrointestinal motility either due to primary qualitative defects of enteric ganglia and nerve fibers, or secondary to a variety of conditions, such as myopathies, inflammatory or autoimmune diseases, drug toxicity, ischemia, or viral infections. CIIPX is diagnosed by radiological, surgical, or manometric evidence of abnormal bowel motility causing intestinal obstruction in the absence of any mechanical occlusion. SIMILARITY: Belongs to the filamin family. & Contains 1 actin-binding domain. & Contains 2 CH (calponin-homology) domains. & Contains 24 filamin repeats.
Molecular Weight	270-280 kDa & 190 kDa

Biological Information

Immunogen

Human platelet protein.

Clone

PM6/317

Concentration

Please refer to the Certificate of Analysis for the lot-specific concentration.

Host

Mouse

Specificity

Recognizes unprocessed, full length Human filamin (actin-binding protein; 270-280 kDa) as well as the 190 kDa N-terminal calpain cleavage fragment of filamin (Aakhus, 1992). Following induction of apoptosis in U937 cells, the antibody recognizes 170, 150, and 120 kDa N-terminal cleavage fragments of the full-length form presumably resulting from cleavage by activated caspase-3 (Umeda, 2001).

Isotype

IgG1

Species Reactivity

Chicken
Guinea Pig
Human
Mouse
Rat
Rabbit

Species Reactivity Note

Human, mouse, and rat. Expected to cross-react with chicken, guinea pig and rabbit.

Antibody Type

Monoclonal Antibody

Entrez Gene Number

NM_001110556.1

Entrez Gene Summary

Actin-binding protein, or filamin, is a 280-kD protein that crosslinks actin filaments into orthogonal networks in cortical cytoplasm and participates in the anchoring of membrane proteins for the actin cytoskeleton. Remodeling of the cytoskeleton is central to the modulation of cell shape and migration. Filamin A, encoded by the FLNA gene, is a widely expressed protein that regulates reorganization of the actin cytoskeleton by interacting with integrins, transmembrane receptor complexes, and second messengers.[supplied by OMIM]

Gene Symbol

FLNA
Alpha-filamin
OPD2
Filamin-A
MNS
FLN
FMD
OPD1
FLN1
NHBP
ABP-280
ABPX
OPD
Filamin-1
DKFZp434P031

Purification Method

Unpurified

UniProt Number

P21333

UniProt Summary

FUNCTION: SwissProt: P21333 # 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).
SIZE: 2647 amino acids; 280739 Da
SUBUNIT: Interacts with PDLIM2 (By similarity). Homodimer. Interacts with 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.
TISSUE SPECIFICITY: Ubiquitous.
DOMAIN: SwissProt: P21333 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. & The N-terminus is blocked.
DISEASE: SwissProt: P21333 # Defects in FLNA are the cause of periventricular nodular heterotopia 1 (PVNH1) [MIM:300049]; also called nodular heterotopia, bilateral periventricular (NHBP or BPNH). PVNH1 is an X-linked developmental dominant disorder in which many neurons fail to migrate into the cerebral cortex. They remain as nodules lining the ventricular surface. In heterozygous females these neurons presumably represent those cells that, after X-chromosome inactivation, contain the active X chromosome with the filamin mutation. Most hemizygous affected males die early during embryogenesis, whereas heterozygous females have normal intelligence but suffer from seizures and various manifestations outside the central nervous system, especially related to the vascular system. This implies that essential embryonic cell migration can only occur in FLNA-expressing cells. & Defects in FLNA are the cause of periventricular nodular heterotopia 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. & 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. & 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. & 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. & 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. & 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. & Defects in FLNA are a cause of X-linked congenital idiopathic intestinal pseudoobstruction (CIIPX) [MIM:300048]. CIIPX is caused by severe abnormality of gastrointestinal motility either due to primary qualitative defects of enteric ganglia and nerve fibers, or secondary to a variety of conditions, such as myopathies, inflammatory or autoimmune diseases, drug toxicity, ischemia, or viral infections. CIIPX is diagnosed by radiological, surgical, or manometric evidence of abnormal bowel motility causing intestinal obstruction in the absence of any mechanical occlusion.
SIMILARITY: Belongs to the filamin family. & Contains 1 actin-binding domain. & Contains 2 CH (calponin-homology) domains. & Contains 24 filamin repeats.

Molecular Weight

270-280 kDa & 190 kDa

Product Usage Statements
Quality Assurance	Routinely evaluated by Western Blot on Jurkat lysates. Western Blot Analysis: 1:500-1:4000 dilution of this lot detected Filamin A on 10 μg of Jurkat lysates. Because of the large size of the unprocessed forms of filamin, 4-7% PAGE gels and proteinase inhibitors are recommended.
Usage Statement	Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Product Usage Statements

Quality Assurance

Routinely evaluated by Western Blot on Jurkat lysates.

Western Blot Analysis:
1:500-1:4000 dilution of this lot detected Filamin A on 10 μg of Jurkat lysates. Because of the large size of the unprocessed forms of filamin, 4-7% PAGE gels and proteinase inhibitors are recommended.

Usage Statement

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Storage and Shipping Information
Storage Conditions	Stable for 1 year at -20°C in undiluted aliquots from date of receipt. Handling Recommendations: Upon first thaw, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.

Storage and Shipping Information

Storage Conditions

Stable for 1 year at -20°C in undiluted aliquots from date of receipt.
Handling Recommendations: Upon first thaw, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.

Packaging Information
Material Size	100 µL

Packaging Information

Material Size

100 µL

实验耗材

分子生物学试剂

蛋白与免疫学

生化试剂

细胞及检测试剂

色谱及层析

仪器设备

实验服务

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