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Rabbit Anti-OTC/Ornithine Carbamoyltransferase  antibody (bs-18037R)
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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-18037R
英文名称 OTC/Ornithine Carbamoyltransferase
中文名称 鸟氨酸氨甲酰基线粒体抗体
别    名 EC 2.1.3.3; MGC129967; MGC129968; MGC138856; OCTD; Ornithine carbamoyltransferase mitochondrial; Ornithine carbamoyltransferase, mitochondrial; Ornithine transcarbamylase; Otc; OTC_HUMAN; OTCase.  
研究领域 细胞生物  免疫学  神经生物学  信号转导  新陈代谢  线粒体  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Rat,Mouse,Human (predicted: Sheep,Horse,Cow,Pig,Dog)
产品应用 WB=1:500-2000, IHC-P=1:100-500, Flow-Cyt=1ug/Test, 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 36kDa
细胞定位 细胞浆 线粒体
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human OTC/Ornithine Carbamoyltransferase: 31-130/354 
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 This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also. [provided by RefSeq, Jul 2008]

Subcellular Location:
Mitochondrion matrix.

Tissue Specificity:
Mainly expressed in liver and intestinal mucosa.

Post-translational modifications:
Acetylation at Lys-88 negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.

DISEASE:
Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD) [MIM:311250]. OTCD is an X-linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the 'neonatal' group (clinical hyperammonemia in the first few days of life) and 'late' onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms.

Similarity:
Belongs to the ATCase/OTCase family.

SWISS:
P00480

Gene ID:
5009

Database links:

Entrez Gene: 280887 Cow

Entrez Gene: 480878 Dog

Entrez Gene: 5009 Human

Entrez Gene: 18416 Mouse

Entrez Gene: 397438 Pig

Entrez Gene: 25611 Rat

Entrez Gene: 101102207 Sheep

Omim: 300461 Human

SwissProt: Q9N1U7 Cow

SwissProt: P00480 Human

SwissProt: P11725 Mouse

SwissProt: O19072 Pig

SwissProt: P00481 Rat

SwissProt: P84010 Sheep

Unigene: 117050 Human

Unigene: 2611 Mouse

Unigene: 2391 Rat



产品图片
Sample:
Liver (Mouse) Lysate at 40 ug
Liver (Rat) Lysate at 40 ug
Primary: Anti- OTC/Ornithine Carbamoyltransferase (bs-18037R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 36 kD
Observed band size: 36 kD
Paraformaldehyde-fixed, paraffin embedded (Rat cerebellum); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (OTC/Ornithine Carbamoyltransferase) Polyclonal Antibody, Unconjugated (bs-18037R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (mouse cerebellum); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (OTC/Ornithine Carbamoyltransferase) Polyclonal Antibody, Unconjugated (bs-18037R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Blank control(black line):HepG2.
Primary Antibody (green line): Rabbit Anti-Ornithine Carbamoyltransferase antibody (bs-18037R)
Dilution:1ug/Test;
Secondary Antibody(white blue line): Goat anti-rabbit IgG-AF488
Dilution: 0.5ug/Test.
Isotype control(orange line): Normal Rabbit IgG
Protocol
The cells were fixed with 4% PFA (10min at room temperature)and then permeabilized with 90% ice-cold methanol for 20 min at -20℃, The cells were then incubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.
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