Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)
浓度
500 ug/ml
产品形态
Liquid
保存条件
Stable for 12 months from date of receipt. Store at -20°C as received.
背景资料
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. [provided by RefSeq, Jul 2008]
研究类别
1. Langley DB, Templeton MD, Fields BA, Mitchell RE, Collyer CA (June 2000). "Mechanism of inactivation of ornithine transcarbamoylase by Ndelta -(N'-Sulfodiaminophosphinyl)-L-ornithine, a true transition state analogue Crystal structure and implications for catalytic mechanism". J. Biol. Chem. 275 (26): 20012–9.2. Nagamine T, Saito S, Kaneko M, Sekiguchi T, Sugimoto H, Takehara K, Takagi H (June 1995). "Effect of biotin on ammonia intoxication in rats and mice". J. Gastroenterol. 30 (3): 351–5.
[list_product_images]Figure 1. Immunohistochemical staining of paraffin-embedded Carcinoma of Human liver tissue using anti-OTC mouse monoclonal antibody. (MA00721) Dilution: 1:150|Figure 2. Immunohistochemical staining of paraffin-embedded Human liver tissue within the normal limits using anti-OTC mouse monoclonal antibody. (MA00721) Dilution: 1:150|Figure 3. HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY OTC (Right lane) cDNA for 48 hrs and lysed. Equivalent amounts of cell lysates (5 ug per lane) were separated by SDS-PAGE and immunoblotted with anti-OTC(Cat# MA00721).[/list_product_images]