Defects in ACTA2 are the cause of aortic aneurysm familial thoracic type 6 (AAT6) [MIM:611788].
AATs are characterized by permanent dilation of the thoracic aorta usually due to degenerative changes in the aortic wall.
They are primarily associated with a characteristic histologic appearance known as 'medial necrosis' or 'Erdheim cystic medial necrosis' in which there is degeneration and fragmentation of elastic fibers, loss of smooth muscle cells, and an accumulation of basophilic ground substance.
Applications:Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence/Immunocytochemistry, Immunoprecipitation, ELISA.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:1000, incubate membrane with diluted primary antibody in TBS, 5% milk, 0.1% Tween 20 at 4°C with gentle shaking, overnight
Immunohistochemistry: 1:50-1:500Immunofluorescence: 1:200Immunoprecipitation: 1:100ELISA (peptide): 1:20,000-1:40,000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：200ul
Host：rabbit
Grade：Affinity Purified
Purity：Purified by affinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of total alpha-SMA. Species Crossreactivity: Mouse, rat and bovine
Isotype：IgG
Calc Applications Abbrev：E IC IF IHC IP WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Synthetic peptide corresponding to N-terminal residues of Human alpha smooth muscle Actin.
