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Rabbit Anti-NAGS  antibody (bs-19004R)
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-19004R
英文名称 NAGS
中文名称 氨基甲酸乙酰转移酶抗体
别    名 1700120E20Rik; AGAS; AI415708; Amino-acid acetyltransferase; ARGA; EC 2.3.1.1; MGC133025; NAGS_HUMAN; N-acetylglutamate synthase; N-acetylglutamate synthase, mitochondrial; RP23-398F7.13.  
研究领域 肿瘤  细胞生物  信号转导  G蛋白信号  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human,Mouse,Rat,Dog,Pig,Cow,Horse,Rabbit)
产品应用 IHC-P=1:100-500, IHC-F=1:100-500, ICC=1:100-500, IF=1:100-500, ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 56kDa
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NAGS: 201-300/534 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C 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.
PubMed PubMed
产品介绍 The N-acetylglutamate synthase gene encodes a mitochondrial enzyme that catalyzes the formation of N-acetylglutamate (NAG) from glutamate and acetyl coenzyme-A. NAG is a cofactor of carbamyl phosphate synthetase I (CPSI), the first enzyme of the urea cycle in mammals. This gene may regulate ureagenesis by altering NAG availability and, thereby, CPSI activity. Deficiencies in N-acetylglutamate synthase have been associated with hyperammonemia. [provided by RefSeq, Jul 2008]

Function:
Plays a role in the regulation of ureagenesis by producing variable amounts of N-acetylglutamate (NAG), thus modulating carbamoylphosphate synthase I (CPSI) activity.

Subcellular Location:
Mitochondrion matrix.

Tissue Specificity:
Highly expressed in the adult liver, kidney and small intestine. Weakly expressed in the fetal liver, lung, pancreas, placenta, heart and brain tissue.

Post-translational modifications:
Probably processed by mitochondrial processing peptidase (MPP). The long form has not yet been isolated

DISEASE:
N-acetylglutamate synthase deficiency (NAGSD) [MIM:237310]: Rare autosomal recessively inherited metabolic disorder leading to severe neonatal or late-onset hyperammonemia without increased excretion of orotic acid. Clinical symptoms are somnolence, tachypnea, feeding difficulties, a severe neurologic presentation characterized by uncontrollable movements, developmental delay, visual impairment, failure to thrive and hyperammonemia precipitated by the introduction of high-protein diet or febrile illness.

Similarity:
Belongs to the acetyltransferase family.
Contains 1 N-acetyltransferase domain.

SWISS:
Q8N159

Gene ID:
162417

Database links:

Entrez Gene: 162417 Human

Entrez Gene: 490943 Dog

Entrez Gene: 217214 Mouse

SwissProt: Q8N159 Human

SwissProt: Q8R4H7 Mouse



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