製品名Anti-CPS1 antibody [EPR7493-3] - BSA and Azide free
CPS1 一次抗体 製品一覧
製品の詳細Rabbit monoclonal [EPR7493-3] to CPS1 - BSA and Azide free
アプリケーション適用あり: ICC/IF, Flow Cyt, IHC-P, IP, WBmore details
種交差性交差種: Mouse, Rat, Human
Synthetic peptide within Human CPS1 aa 100-200. The exact sequence is proprietary.
- WB: HeLa cell lysate and human liver tissue lysate.
ab248283 is the carrier-free version of ab129076. This format is designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.
Our carrier-free formats are supplied in a buffer free of BSA, sodium azide and glycerol for higher conjugation efficiency.
Use our conjugation kits for antibody conjugates that are ready-to-use in as little as 20 minutes with <1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.
ab248283 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.
Maxpar® is a trademark of Fluidigm Canada Inc.
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
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
保存方法Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Concentration information loading...
Our Abpromise guarantee covers the use of ab248283 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||Use at an assay dependent concentration.|
|Flow Cyt||Use at an assay dependent concentration.|
|IHC-P||Use at an assay dependent concentration. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.|
|IP||Use at an assay dependent concentration.|
|WB||Use at an assay dependent concentration. Detects a band of approximately 150 kDa (predicted molecular weight: 165 kDa).|
機能Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
組織特異性Primarily in the liver and small intestine.
関連疾患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.
配列類似性Contains 2 ATP-grasp domains.
Contains 1 glutamine amidotransferase type-1 domain.
ドメインThe type-1 glutamine amidotransferase domain is defective.
- Information by UniProt
- Carbamoyl phosphate synthase [ammonia] antibody
- Carbamoyl phosphate synthase [ammonia] mitochondrial antibody
- Carbamoyl phosphate synthase antibody
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 kDa
This data was developed using the same antibody clone in a different buffer formulation (ab129076).
ab129076 was shown to react with CPS1 in wild-type HeLa cells in Western blot. Loss of signal was observed when knockout sample ab257121 was used. Wild-type HeLa and CPS1 knockout whole cell lysates were subjected to SDS-PAGE. ab129076 and Anti-GAPDH antibody [6C5] - Loading Control (ab8245) overnight at 4°C at a 1 in 1000 dilution and a 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 20000 dilution for 1 hour at room temperature before imaging.
ab248283 has not yet been referenced specifically in any publications.