• 靶点：
• Actinin-α2/3

• 简介：
• >>Arrhythmogenic right ventricular cardiomyopathy

• 基因名称：
• ACTN2/ACTN3

• 蛋白名称：
• Alpha-actinin-2/3

• Human Gene Id：
• 88/89

• Human Swiss Prot No：
• P35609/Q08043

• Mouse Gene Id：
• 11472/11474

• 免疫原：
• The antiserum was produced against synthesized peptide derived from human Actinin alpha-2/3. AA range:31-80

• 特异性：
• Actinin-α2/3 Polyclonal Antibody detects endogenous levels of Actinin-α2/3 protein.

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

• 来源：
• Polyclonal, Rabbit,IgG

• 稀释：
• WB 1:500 - 1:2000. IHC 1:100 - 1:300. IF 1:200 - 1:1000. ELISA: 1:20000. Not yet tested in other applications.

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

• 浓度：
• 1 mg/ml

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

• 其他名称：
• ACTN2;Alpha-actinin-2;Alpha-actinin skeletal muscle isoform 2;F-actin cross-linking protein;ACTN3;Alpha-actinin-3;Alpha-actinin skeletal muscle isoform 3;F-actin cross-linking protein

• 实测条带：
• 103kD

• 背景：
• 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],

• 功能：
• disease: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 r

• 细胞定位：
• Cytoplasm, myofibril, sarcomere, Z line . Colocalizes with MYOZ1 and FLNC at the Z-lines of skeletal muscle.

• 组织表达：
• Expressed in both skeletal and cardiac muscle.

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