Product Specification
Host |
Rabbit |
Antigen |
alpha-actinin-2 |
Synonyms |
ACTN2 |
Immunogen |
Synthetic Peptide |
Location |
Cytoplasm |
Accession |
P35609 |
Clone Number |
SDT-032-79 |
Antibody Type |
Rabbit mAb |
Application |
WB, IHC-P, IP |
Reactivity |
Hu, Ms, Rt |
Predicted Reactivity |
Bv |
Purification |
Protein A |
Concentration |
0.25 mg/ml |
Physical Appearance |
Liquid |
Storage Buffer |
PBS, 40% Glycerol, 0.05%BSA, 0.03% Proclin 300 |
Stability & Storage |
12 months from date of receipt / reconstitution, -20 °C as supplied |
Dilution
application |
dilution |
species |
WB |
1:500 |
|
IHC-P |
1:1000 |
|
IP |
1:25 |
|
Background
Alpha-actinin-2 is a protein which in humans is encoded by the ACTN2 gene. This gene encodes an alpha-actinin isoform that is expressed in both skeletal and cardiac muscles and functions to anchor myofibrillar actin thin filaments and titin to Z-discs. The primary function of alpha-actinin-2 is to crosslink filamentous actin molecules and titin molecules from adjoining sarcomeres at Z-discs, a function that is modulated by phospholipids. Mutations in ACTN2 are associated with hypertrophic cardiomyopathy, as well as dilated cardiomyopathy and endocardial fibroelastosis. The diverse functions of alpha-actinin-2 are reflected in the diverse clinical presentation of patients carrying ACTN2 mutations.