SKU: WM0470 Categories: ,

Actinin-α2 Monoclonal Antibody

Price range: $268.00 through $328.00

SKU: WM0470-50
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Product details

Background:
Alpha actinins belong to the spectrin gene superfamily which represents a diverse group of cytoskeletal proteins, including the alpha and beta spectrins and dystrophins. Alpha actinin is an actin-binding protein with multiple roles in different cell types. In nonmuscle cells, the cytoskeletal isoform is found along microfilament bundles and adherens-type junctions, where it is involved in binding actin to the membrane. In contrast, skeletal, cardiac, and smooth muscle isoforms are localized to the Z-disc and analogous dense bodies, where they help anchor the myofibrillar actin filaments. This gene encodes a muscle-specific, alpha actinin isoform that is expressed in both skeletal and cardiac muscles. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2013],

Specifications

Size50μL/100μL
TargetActinin-α2
ReactivityHuman, Rat, Bovine, Chicken, Dog, Pig, Zebrafish
ApplicationsWB, IF
MW(Calculated)104kD
MW(Observed)
Host SpeciesMouse
Isotype
Conjugate/ModificationUnmodified
Recommended Dilution RatioWB 1:1000-1:2000; IF 1:100-1:500; Not yet tested in other applications.
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
PurificationAffinity purification
Storage-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
ClonalityMonoclonal
Clone Number6H8
ImmunogenPurified recombinant human Actinin-α2 (C-terminus) protein fragments expressed in E.coli.
SpecificityActinin-α2 Monoclonal Antibody detects endogenous levels of Actinin-α2 protein.
Gene NameACTN2/ACTN3
Protein NameAlpha-actinin-2
Other NameACTN2; / Alpha-actinin-2; / Alpha-actinin skeletal muscle isoform 2; / F-actin cross-linking protein
SpeciesHuman
Gene ID88
UniprotP35609,
Species.1Mouse
Gene ID.1
Uniprot.1Q9JI91
Species.2
Gene ID.2
Uniprot.2
Cellular LocalizationCytoplasm, myofibril, sarcomere, Z line . Colocalizes with MYOZ1 and FLNC at the Z-lines of skeletal muscle.
Tissue ExpressionExpressed in both skeletal and cardiac muscle.
Signaling_pathway
Research Areas>>Arrhythmogenic right ventricular cardiomyopathy
FunctionDisease:Defects in ACTN2 are the cause of cardiomyopathy dilated type 1AA (CMD1AA) [MIM:612158]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.,Function:F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein.,similarity:Belongs to the alpha-actinin family.,similarity:Contains 1 actin-binding domain.,similarity:Contains 2 CH (calponin-homology) domains.,similarity:Contains 2 EF-hand domains.,similarity:Contains 4 spectrin repeats.,subcellular location:Colocalizes with MYOZ1 and FLNC at the Z-lines of skeletal muscle.,subunit:Homodimer; antiparallel. Also forms heterodimers with ACTN3. Interacts with ADAM12, MYOZ1, MYOZ2 and MYOZ3. Interacts via its C-terminal region with the LDB3 PDZ domain. Interacts with XIRP2.,tissue specificity:Expressed in both skeletal and cardiac muscle.,
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