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Rabbit Anti-NAGS/BF594 Conjugated antibody (bs-19004R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-19004R-BF594
英文名稱1 Rabbit Anti-NAGS/BF594 Conjugated antibody
中文名稱 BF594標記的氨基甲酸乙酰轉移酶抗體
別    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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  信號轉導  G蛋白信號  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應
產品應用 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 56kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NAGS
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 background:
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.

Database links:

Entrez Gene: 162417 Human

Entrez Gene: 490943 Dog

Entrez Gene: 217214 Mouse

SwissProt: Q8N159 Human

SwissProt: Q8R4H7 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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