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 Immunofluorescence, Western Blot and Immunohistochemistry.
Other applications not tested.
Recommended Dilutions:Western Blot: 1:1000Immunohistochemistry: 1:50-1:200Immunofluorescence: 1:50-1:200Optimal 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：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography
Form：Supplied as a liquid PBS, pH 7.2, 0.02% sodium azide, 50% glycerol
Specificity：Recognizes endogenous levels of human alpha-SMA. Species Crossreactivity: mouse and rat
Isotype：IgG
Calc Applications Abbrev：IF IHC WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Full length recombinant protein corresponding to human α-SMA.
