OTC/Ornithine Carbamoyltransferase Rabbit pAb (一抗) | Bioss
货号：bs-18037R
产品详情
相关标记
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常见问题
概述
产品编号
bs-18037R
产品类型
宠物抗体、农牧业/家禽抗体
英文名称
OTC/Ornithine Carbamoyltransferase Rabbit pAb
中文名称
鸟氨酸氨甲酰基线粒体抗体
英文别名
OCTD; OTC1; OTCD; OTCase; Sf; spf; OTC_BOVIN; OTC; Ornithine carbamoyltransferase, mitochondrial; 2.1.3.3; OTC_HUMAN; OTC_MOUSE; OTC_PIG; OTC_RAT; OTC_SHEEP; ornithine transcarbamylase; ornithine carbamoyltransferase; ornithine transcarbamylase deficiency
抗体来源
Rabbit
免疫原
KLH conjugated synthetic peptide derived from human OTC/Ornithine Carbamoyltransferase: 31-130/354
亚型
IgG
性状
Liquid
纯化方法
affinity purified by Protein A
克隆类型
Polyclonal
理论分子量
36 kDa
检测分子量
36 kDa
浓度
1mg/ml
储存液
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
研究领域
Metabolism > Pathways and Processes > Metabolic signaling pathways > Amino acid metabolism
Metabolism > Pathways and Processes > Mitochondrial Metabolism > Mitochondrial markers
Signal Transduction > Metabolism > Amino Acids
Signal Transduction > Metabolism > Mitochondrial
Tags & Cell Markers > Subcellular Markers > Organelles > Mitochondria
SWISS
Gene ID
保存条件
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
数据库链接
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]
产品应用
| 应用 | 已检合格种属 | 预测种属 | 推荐稀释比例 |
|---|---|---|---|
| WB | Mouse | Human, Rat, Pig, Sheep, Cow, Dog, Horse | 1:500-2000 |
| IHC-P | Mouse, Rat | Human, Pig, Sheep, Cow, Dog, Horse | 1:100-500 |
| IHC-F | Mouse, Rat | Human, Pig, Sheep, Cow, Dog, Horse | 1:100-500 |
| IF | Mouse, Rat | Human, Pig, Sheep, Cow, Dog, Horse | 1:100-500 |
| Flow-Cyt | Human | Mouse, Rat, Pig, Sheep, Cow, Dog, Horse | 1ug/Test |
交叉反应
交叉反应: Human, Mouse, Rat (predicted: Pig, Sheep, Cow, Dog, Horse)
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靶标
基因名
OTC
蛋白名
Ornithine carbamoyltransferase, mitochondrial
亚基
Homotrimer.
亚细胞定位
Mitochondrion matrix.
组织特异性
Mainly expressed in liver and intestinal mucosa.
翻译后修饰
Acetylation at Lys-88 negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.
疾病
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.
相似性
Belongs to the ATCase/OTCase family.
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