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NAGS rabbit pAb

商品貨號： PLA020272

適應(yīng) 性： 人,小鼠

￥600元

規(guī)格：

40μL 100μL 200μL

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MSDS

說明書

商品描述

基因名稱： NAGS
蛋白名稱： NAGS
Human_gene_id： 162417
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=162417
Human_swiss_prot_no： Q8N159
Human_swiss_link： https://www.uniprot.org/uniprotkb/Q8N159/entry
Mouse_gene_id： 217214
Mouse_gene_link： https://www.uniprot.org/uniprot/217214
Mouse_swiss_prot_no： Q8R4H7
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/Q8R4H7
特異性： This antibody detects endogenous levels of NAGS at Human/Mouse
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來源： Polyclonal, Rabbit,IgG
稀釋： WB 1:500-2000;IHC-p 1:50-300
純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度： 1 mg/ml
儲存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
分子量： 59kD
功能： catalytic activity:Acetyl-CoA + L-glutamate = CoA + N-acetyl-L-glutamate.,disease:Defects in NAGS are the cause of N-acetylglutamate synthase deficiency (NAGSD) [MIM:237310]. NAGSD is a 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.,enzyme regulation:Increased by L-arginine.,function:Plays a role in the regulation of ureagenesis by producing variable amounts of N-acetylglutamate (NAG), thus modulating carbamoylphosphate synthase I (CPSI) activity.,online information:N-acetylglutamate synthase entry,pathway:Amino-acid biosynthesis; L-arginine biosynthesis; N(2)-acetyl-L-ornithine from L-glutamate: step 1/4.,PTM:Probably processed by mitochondrial processing peptidase (MPP). The long form has not yet been isolated.,similarity:Belongs to the acetyltransferase family.,similarity:Contains 1 N-acetyltransferase domain.,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.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： Mitochondrion matrix .
組織表達(dá)： Highly expressed in the adult liver, kidney and small intestine. Weakly expressed in the fetal liver, lung, pancreas, placenta, heart and brain tissue.
科研貨號： PLA020272

NAGS rabbit pAb

Catalog No PLA020272

Product information

基因名稱： NAGS
蛋白名稱： NAGS
Human_gene_id： 162417
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=162417
Human_swiss_prot_no： Q8N159
Human_swiss_link： https://www.uniprot.org/uniprotkb/Q8N159/entry
Mouse_gene_id： 217214
Mouse_gene_link： https://www.uniprot.org/uniprot/217214
Mouse_swiss_prot_no： Q8R4H7
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/Q8R4H7
特異性： This antibody detects endogenous levels of NAGS at Human/Mouse
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來源： Polyclonal, Rabbit,IgG
稀釋： WB 1:500-2000;IHC-p 1:50-300
純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度： 1 mg/ml
儲存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
分子量： 59kD
功能： catalytic activity:Acetyl-CoA + L-glutamate = CoA + N-acetyl-L-glutamate.,disease:Defects in NAGS are the cause of N-acetylglutamate synthase deficiency (NAGSD) [MIM:237310]. NAGSD is a 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.,enzyme regulation:Increased by L-arginine.,function:Plays a role in the regulation of ureagenesis by producing variable amounts of N-acetylglutamate (NAG), thus modulating carbamoylphosphate synthase I (CPSI) activity.,online information:N-acetylglutamate synthase entry,pathway:Amino-acid biosynthesis; L-arginine biosynthesis; N(2)-acetyl-L-ornithine from L-glutamate: step 1/4.,PTM:Probably processed by mitochondrial processing peptidase (MPP). The long form has not yet been isolated.,similarity:Belongs to the acetyltransferase family.,similarity:Contains 1 N-acetyltransferase domain.,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.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： Mitochondrion matrix .
組織表達(dá)： Highly expressed in the adult liver, kidney and small intestine. Weakly expressed in the fetal liver, lung, pancreas, placenta, heart and brain tissue.
科研貨號： PLA020272

Hunan UPT Biotechnology Co.,Ltd

Website：m.tjdqdt.com Servive hotline :4006916686

E-mail：service@uptbio.com

Address：

Room 402, Building 13, Xinggong International Industrial Park, 100 Guyuan Road, Yuelu District, Changsha City, Hunan Province, China.

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