The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
IHC-P: 1/100 - 1/250. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
WB: 1/20000 - 1/50000. Detects a band of approximately 103 kDa (predicted molecular weight: 103 kDa).
Is unsuitable for Flow Cyt or ICC/IF.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein.
Expressed in both skeletal and cardiac muscle.
Involvement in 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.