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货号： bs-18037R-Cy5.5 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-18037R-Cy5.5
- 英文名称
- Anti-OTC/Ornithine Carbamoyltransferase/Cy5.5
- 中文名称
- Cy5.5标记的鸟氨酸氨甲酰基线粒体抗体
- 别 名
- EC 2.1.3.3; MGC129967; MGC129968; MGC138856; OCTD; Ornithine carbamoyltransferase mitochondrial; Ornithine carbamoyltransferase, mitochondrial; Ornithine transcarbamylase; Otc; OTC_HUMAN; OTCase.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 免疫学 神经生物学 信号转导 新陈代谢 线粒体
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 36kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human OTC/Ornithine Carbamoyltransferase
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
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]
Subcellular Location:
Mitochondrion matrix.
Tissue Specificity:
Mainly expressed in liver and intestinal mucosa.
Post-translational modifications:
Acetylation at Lys-88 negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.
DISEASE:
Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD) [MIM:311250]. OTCD is an X-linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the neonatal group (clinical hyperammonemia in the first few days of life) and late onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms.
Similarity:
Belongs to the ATCase/OTCase family.
Database links:Entrez Gene: 280887Cow
Entrez Gene: 480878Dog
Entrez Gene: 5009Human
Entrez Gene: 18416Mouse
Entrez Gene: 397438Pig
Entrez Gene: 25611Rat
Entrez Gene: 101102207Sheep
Omim: 300461Human
SwissProt: Q9N1U7Cow
SwissProt: P00480Human
SwissProt: P11725Mouse
SwissProt: O19072Pig
SwissProt: P00481Rat
SwissProt: P84010Sheep
Unigene: 117050Human
Unigene: 2611Mouse
Unigene: 2391Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.