OTC (Ornithine Carbamoyltransferase, Mitochondrial, Ornithine Carbamoyltransferase)

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£599.00

SKU:: 487853
Reinheit:: Purified by Protein A affinity chromatography.
Lagerbedingungen:: -20°C
Hersteller:: United States Biological
Host:: Rabbit
Immunogen:: Synthetic peptide corresponding to the N-terminal region of human OTC
Format:: Supplied as a liquid in PBS, 2% sucrose, 0.09% sodium azide.
Weitere Details:: OTC is 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.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 Immunohistochemistry, Western Blot. Other applications not tested. Recommended Dilution: Optimal 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.