• Target：
• Actinin-α3

• Fields：
• >>Arrhythmogenic right ventricular cardiomyopathy

• Gene Name：
• ACTN3

• Protein Name：
• Alpha-actinin-3

• Human Gene Id：
• 89

• Human Swiss Prot No：
• Q08043

• Mouse Gene Id：
• 11474

• Mouse Swiss Prot No：
• O88990

• Immunogen：
• The antiserum was produced against synthesized peptide derived from human ACTN3. AA range:1-50

• Specificity：
• Actinin-α3 Polyclonal Antibody detects endogenous levels of Actinin-α3 protein.

• Formulation：
• Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

• Source：
• Polyclonal, Rabbit,IgG

• Dilution：
• WB 1:500 - 1:2000. IF 1:200 - 1:1000. ELISA: 1:20000. Not yet tested in other applications.

• Purification：
• The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

• Concentration：
• 1 mg/ml

• Storage Stability：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• Other Name：
• ACTN3;Alpha-actinin-3;Alpha-actinin skeletal muscle isoform 3;F-actin cross-linking protein

• Observed Band(KD)：
• 103kD

• Background：
• This gene encodes a member of the alpha-actin binding protein gene family. The encoded protein is primarily expressed in skeletal muscle and functions as a structural component of sarcomeric Z line. This protein is involved in crosslinking actin containing thin filaments. An allelic polymorphism in this gene results in both coding and non-coding variants; the reference genome represents the coding allele. The non-functional allele of this gene is associated with elite athlete status. [provided by RefSeq, Feb 2014],

• Function：
• function:F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein.,polymorphism:About 18% of the world population lack a functional ACTN3 due to a stop codon polymorphism at position 577. The absence of a functional ACTN3 expression is not correlated with a disease state.,sequence caution:According to the human genome assembly there is a stop codon in position 577 which is only found in 18% of the world population.,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.,subunit:Homodimer; antiparallel. Also forms heterodimers with ACTN2. Interacts with MYOZ1.,tissue specificity:Expressed only in a subset of type 2 skeletal muscle fibers.,

• Subcellular Location：
• intracellular,cytosol,actin filament,brush border,focal adhesion,Z disc,pseudopodium,extracellular exosome,

• Expression：
• Expressed only in a subset of type 2 skeletal muscle fibers.

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