Molecular weight (DA)
103kD
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.
Dilution rate
Western Blot: 1/500 - 1/2000. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/20000. Not yet tested in other applications.
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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.,
Protein name
Alpha-actinin-3
Other name
ACTN3; Alpha-actinin-3; Alpha-actinin skeletal muscle isoform 3; F-actin cross-linking protein
Fields
>>Arrhythmogenic right ventricular cardiomyopathy
Human protein sequence Database
Q08043
Mouse protein sequence database
O88990
Rat protein sequence database
Cellular localization
intracellular,cytosol,actin filament,brush border,focal adhesion,Z disc,pseudopodium,extracellular exosome,
Tissue expression
Expressed only in a subset of type 2 skeletal muscle fibers.
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