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Anti-Actinin-α2/3 (Q58) Antibody

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产品名称: Anti-Actinin-α2/3 (Q58) Antibody
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Anti-Actinin-α2/3 (Q58) Antibody


Anti-Actinin-α2/3 (Q58) Antibody  的详细介绍
Name: Anti-Actinin-α2/3 (Q58) Antibody
See all Actinin-α2 primary antibodies
Description: Rabbit polyclonal antibody to Actinin-α2/3 (Q58)
Specificity: Actinin-α2/3 (Q58) pAb detects endogenous levels of actinin-α2 and actinin-α3 protein.
Applications: WB, IHC, IF
Reactivity: Human, Mouse, Rat
Immunogen: Synthetic peptide, corresponding to amino acids 30-70 of Human Actinin-α2.
Host: Rabbit
Clonality: Polyclonal
Conjugate: Unconjugated
Molecular Weight: ~ 103 kDa
Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Product Form: 1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.
Function: F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein.
Tissue Specificity: Expressed in both skeletal and cardiac muscle.
Involvement in Disease: Cardiomyopathy, familial hypertrophic 23, with or without left ventricular non-compaction: A hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death.

Cardiomyopathy, dilated 1AA, with or without left ventricular non-compaction: A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
Sequence Similarities: Belongs to the alpha-actinin family.
Post-Translational Modification: Ubiquitinated by FBXL22, leading to proteasomal degradation.
Cellular Location: Cytoplasm > Myofibril > Sarcomere > Z line.

Colocalizes with MYOZ1 and FLNC at the Z-lines of skeletal muscle.
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