重组Anti-CPS1抗体[EPR7493-3] (ab129076)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR7493-3] to CPS1
- Suitable for: Flow Cyt (Intra), WB, IHC-P
- Knockout validated
- Reacts with: Mouse, Rat, Human
Related conjugates and formulations
概述
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产品名称
Anti-CPS1抗体[EPR7493-3]
参阅全部 CPS1 一抗 -
描述
兔单克隆抗体[EPR7493-3] to CPS1 -
宿主
Rabbit -
经测试应用
适用于: Flow Cyt (Intra), WB, IHC-Pmore details -
种属反应性
与反应: Mouse, Rat, Human -
免疫原
Synthetic peptide within Human CPS1 aa 100-200. The exact sequence is proprietary.
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阳性对照
- WB: Human fetal liver, mouse liver and rat liver tissue lysates and HeLa cell lysate. IHC-P: Human liver tissue. Flow Cyt (intra): HeLa cells.
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常规说明
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C. -
存储溶液
pH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA, 50% Tissue culture supernatant -
Concentration information loading...
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纯度
Protein A purified -
克隆
单克隆 -
克隆编号
EPR7493-3 -
同种型
IgG -
研究领域
相关产品
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Alternative Versions
- HRP Anti-CPS1 antibody [EPR7493-3] (ab198969)
- Anti-CPS1 antibody [EPR7493-3] - BSA and Azide free (ab248283)
- Alexa Fluor® 488 Anti-CPS1 antibody [EPR7493-3] (ab252114)
- Alexa Fluor® 647 Anti-CPS1 antibody [EPR7493-3] (ab252115)
- APC Anti-CPS1 antibody [EPR7493-3] (ab319330)
- PE Anti-CPS1 antibody [EPR7493-3] (ab319467)
- Alexa Fluor® 594 Anti-CPS1 antibody [EPR7493-3] (ab319823)
- Alexa Fluor® 555 Anti-CPS1 antibody [EPR7493-3] (ab319964)
- Alexa Fluor® 750 Anti-CPS1 antibody [EPR7493-3] (ab321026)
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Compatible Secondaries
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Isotype control
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KO cell lines
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KO cell lysates
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Positive Controls
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab129076于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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Flow Cyt (Intra) |
1/20.
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WB |
1/1000 - 1/10000. Detects a band of approximately 150 kDa (predicted molecular weight: 165 kDa).
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IHC-P |
1/100 - 1/250. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
|
说明 |
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Flow Cyt (Intra)
1/20. |
WB
1/1000 - 1/10000. Detects a band of approximately 150 kDa (predicted molecular weight: 165 kDa). |
IHC-P
1/100 - 1/250. Perform heat mediated antigen retrieval before commencing with IHC staining protocol. |
靶标
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功能
Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell. -
组织特异性
Primarily in the liver and small intestine. -
疾病相关
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 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. -
序列相似性
Contains 2 ATP-grasp domains.
Contains 1 glutamine amidotransferase type-1 domain. -
结构域
The type-1 glutamine amidotransferase domain is defective. -
细胞定位
Mitochondrion. - Information by UniProt
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数据库链接
- Entrez Gene: 1373 Human
- Entrez Gene: 227231 Mouse
- Entrez Gene: 497840 Rat
- Omim: 608307 Human
- SwissProt: P31327 Human
- SwissProt: Q8C196 Mouse
- SwissProt: P07756 Rat
- Unigene: 149252 Human
see all -
别名
- Carbamoyl phosphate synthase [ammonia] antibody
- Carbamoyl phosphate synthase [ammonia] mitochondrial antibody
- Carbamoyl phosphate synthase antibody
see all
图片
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All lanes : Anti-CPS1 antibody [EPR7493-3] (ab129076) at 1/1000 dilution
Lane 1 : Wild-type HeLa cell lysate
Lane 2 : CPS1 knockout HeLa cell lysate
Lane 3 : Human liver tissue lysate
Lysates/proteins at 20 µg per lane.
Performed under reducing conditions.
Predicted band size: 165 kDa
Observed band size: 165 kDaLanes 1-3: Merged signal (red and green). Green - ab129076 observed at 165 kDa. Red - loading control, ab8245 observed at 37 kDa.
ab129076 Anti-CPS1 antibody [EPR7493-3] was shown to specifically react with CPS1 in wild-type HeLa cells. Loss of signal was observed when knockout cell line ab261809 (knockout cell lysate ab257121) was used. Wild-type and CPS1 knockout samples were subjected to SDS-PAGE. ab129076 and Anti-GAPDH antibody [6C5] - Loading Control (ab8245) were incubated overnight at 4°C at 1 in 1000 dilution and 1 in 20000 dilution respectively. Blots were developed with Goat anti-Rabbit IgG H&L (IRDye® 800CW) preadsorbed (ab216773) and Goat anti-Mouse IgG H&L (IRDye® 680RD) preadsorbed (ab216776) secondary antibodies at 1 in 10000 dilution for 1 hour at room temperature before imaging.
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All lanes : Anti-CPS1 antibody [EPR7493-3] (ab129076) at 1/1000 dilution
Lane 1 : Fetal liver cell lysate
Lane 2 : Mouse liver cell lysate
Lane 3 : Rat liver cell lysate
Lane 4 : HeLa cell lysate
Predicted band size: 165 kDa
Observed band size: 150 kDa why is the actual band size different from the predicted? -
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-CPS1 antibody [EPR7493-3] (ab129076)
ab129076, at 1/100 dilution staining CPS1 in formalin fixed paraffin embedded Human liver tissue by immunohistochemistry
Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
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Intracellular Flow Cytometry analysis of HeLa (Human cervix adenocarcinoma epithelial cell) cells labeling CPS1 (red) with ab129076 at a 1/20 dilution. Cells were fixed with 4% paraformaldehyde and permeabilized with 90% methanol. A goat anti-rabbit IgG (Alexa Fluorr® 488) (ab150077) was used as the secondary antibody at a 1/2000 dilution. Black - Rabbit monoclonal IgG (Black) (ab172730). Blue (unlabeled control) - Cell without incubation with primary antibody and secondary antibody (Blue).
数据表及文件
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SDS download
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Datasheet download
文献 (11)
ab129076 被引用在 11 文献中.
- Wang ZX et al. Limited role for hyperammonemia in the progression of diet-induced nonalcoholic steatohepatitis. J Dig Dis 24:408-418 (2023). PubMed: 37529891
- Cabanes-Creus M et al. Characterization of the humanized FRG mouse model and development of an AAV-LK03 variant with improved liver lobular biodistribution. Mol Ther Methods Clin Dev 28:220-237 (2023). PubMed: 36700121
- Missiaen R et al. GCN2 inhibition sensitizes arginine-deprived hepatocellular carcinoma cells to senolytic treatment. Cell Metab 34:1151-1167.e7 (2022). PubMed: 35839757
- Scheiter A et al. RASSF1A independence and early galectin-1 upregulation in PIK3CA-induced hepatocarcinogenesis: new therapeutic venues. Mol Oncol 16:1091-1118 (2022). PubMed: 34748271
- Forcina GC et al. Ferroptosis regulation by the NGLY1/NFE2L1 pathway. Proc Natl Acad Sci U S A 119:e2118646119 (2022). PubMed: 35271393