氨甲酰磷酸合成酶1抗体
规格:1尘驳/1尘濒
英文名: CPS1
别名: Carbamoyl phosphate synthase [ammonia]; Carbamoyl phosphate synthase [ammonia] mitochondrial; Carbamoyl phosphate synthase; Carbamoyl phosphate synthetase 1; Carbamoyl phosphate synthetase 1 mitochond
分子量: 160kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:笔辞濒测肠濒辞苍补濒
亚型:滨驳骋
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human CPS1
交叉反应:Human, Mouse, Rat, Dog, Sheep,
细胞定位:
氨甲酰磷酸合成酶1抗体产物介绍:background: 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] Function: Involved in the urea cycle of ureotelic animals where the enzyme plays 氨甲酰磷酸合成酶1抗体an important role in removing excess ammonia from the cell. Subcellular Location: Mitochondrion. Tissue Specificity: Primarily in the liver and small intestine. DISEASE: 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 氨甲酰磷酸合成酶1抗体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. Similarity: Contains 2 ATP-grasp domains. Contains 1 glutamine amidotransferase type-1 domain. Gene ID: 1373 Database links: Entrez Gene: 1373 Human Omim: 608307 Human SwissProt: P31327 Human Unigene: 149252 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
氨甲酰磷酸合成酶1抗体产物应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域:肿瘤 细胞生物 神经生物学 信号转导 细胞类型标志物 新陈代谢 线粒体
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid