CPS1 Recombinant Rabbit mAb (一抗) - WB,IHC-P,IHC-F,IF | Bioss
Rrmab?兔单抗
货号：bsm-60596R
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概述
产品编号
bsm-60596R
产品类型
重组兔单抗
英文名称
CPS1 Recombinant Rabbit mAb
中文名称
氨甲酰磷酸合成酶1重组兔单抗
英文别名
CPSASE1; GATD6; PHN; CPSM_HUMAN; CPS1; Carbamoyl-phosphate synthetase I (CPSase I); 6.3.4.16;
抗体来源
Rabbit
亚型
IgG
性状
Liquid
纯化方法
affinity purified by Protein A
克隆类型
Recombinant
克隆号
C6G2
检测分子量
165 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
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.
背景资料
The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This reaction is the first committed step of the urea cycle, which is important in the removal of excess urea from cells. The encoded protein may also represent a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been found for this gene. The shortest isoform may not be localized to the mitochondrion. Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation.[provided by RefSeq, May 2010]
产品应用
| 应用 | 已检合格种属 | 预测种属 | 推荐稀释比例 |
|---|---|---|---|
| WB | Human | Mouse, Rat | 1:500-1000 |
| IHC-P | Human | Mouse, Rat | 1:100-500 |
| IHC-F | Human | Mouse, Rat | 1:100-500 |
| IF | Human | Mouse, Rat | 1:100-500 |
交叉反应
交叉反应: Human (predicted: Mouse, Rat)
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靶标
基因名
CPS1
蛋白名
Carbamoyl-phosphate synthase [ammonia], mitochondrial
亚细胞定位
Mitochondrion.
组织特异性
Primarily in the liver and small intestine.
疾病
Defects in CPS1 are the cause of carbamoyl phosphate synthetase 1 deficiency (CPS1D) [MIM:237300]. CPS1D is an autosomal recessive disorder of the urea cycle causing hyperammonemia. Clinical features include protein intolerance, intermittent ataxia, seizures, lethargy, developmental delay and mental retardation.
Note=Genetic variations in CPS1 influence the availability of precursors for nitric oxide (NO) synthesis and play a role in clinical situations where endogenous NO production is critically important, such as neonatal pulmonary hypertension, increased pulmonary artery pressure following surgical repair of congenital heart defects or hepatovenocclusive disease following bone marrow transplantation. Infants with neonatal pulmonary hypertension homozygous for Thr-1406 have lower L-arginine concentrations than neonates homozygous for Asn-1406.
相似性
Contains 2 ATP-grasp domains.
Contains 1 glutamine amidotransferase type-1 domain.
Contains 1 glutamine amidotransferase type-1 domain.
功能
Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
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