This nuclear gene encodes a mitochondrial matrix enzyme.
Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia.
Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also.
Applications:Suitable for use in ELISA, Western Blot and Immunohistochemistry.
Other applications not tested.
Recommended Dilution:ELISA: 1:2000-1:5000Western Blot: 1:500-1:2000Immunohistochemistry: 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 in PBS, pH 7.4, 0.05% sodium azide, 40% glycerol.
Specificity：Species Crossreactivity: mouse and rat
