Biolegend, 630752, Purified anti-CPS1 Antibody, 100μg

CPS1, or carbamoyl phosphate synthase 1, catalyzes the initial step in the urea cycle to produce carbamoyl phosphate from ammonia and bicarbonate. CPS1 is highly expressed in the mitochondria of hepatocytes, where a robust urea cycle takes place to detoxify ammonia from protein catabolism. CPS1 deficiency in humans is an autosomal recessive disease that causes severe symptoms, such as hyperammonemia, in urea cycle disorders. This deficiency affects the neuronal and metabolic development of newborn children. CPS1 undergoes various post-translational modifications, including acetylation, succinylation, and glutarylation, which regulate the enzymatic activity of CPS1. During fasting, sirtuin 5 (SIRT5) is activated by increased NAD levels, leading to the deacetylation and deglutarylation of CPS1. This process removes inhibitory modifications from the active ATP binding site. The tumor suppressor p53 regulates ammonia metabolism by repressing the urea cycle and the expression of genes such as CPS1, OTC, and ARG1. The repression leads to the inhibition of ureagenesis and the elimination of ammonia, ultimately impacting tumor growth.
100μg
Verified Reactivity: Human, Mouse
Antibody Type: Monoclonal
Host Species: Rat
Immunogen: Recombinant fragment of human CPS1
Formulation: Phosphate-buffered solution, pH 7.2, containing 0.09% sodium azide
Preparation: The antibody was purified by affinity chromatography.
Concentration: 0.5 mg/mL
Storage & Handling: The antibody solution should be stored undiluted between 2°C and 8°C.
Application: WB - Quality tested ICC, IHC-P - Verified
Recommended Usage: Each lot of this antibody is quality control tested by western blotting. For western blotting, the suggested use of this reagent is 0.25 - 1.0 µg/mL. For immunocytochemistry, a concentration range of 1.0 - 10.0 μg/mL is recommended. For immunohistochemistry on formalin-fixed paraffin-embedded tissue sections, a concentration range of 0.25 - 1.0 µg/mL is suggested. It is recommended that the reagent be titrated for optimal performance for each application.
Application Notes: For immunocytochemistry (ICC), the recommended fixation/permeabilization method is either 4% paraformaldehyde/0.5% Triton X-100, paraformaldehyde followed by ice-cold methanol, or ice-cold methanol fixed only. For immunohistochemistry (IHC), the recommended antigen retrieval system is either Citrate Buffer, 10X (Cat. No. 420902) or Tris-EDTA pH 9.0 buffer.
RRID: AB_3083419 (BioLegend Cat. No. 630751) AB_3083419 (BioLegend Cat. No. 630752)
Structure: 3 isoforms exist/form homodimer and homooligomer
Distribution: Liver and small intestine/mitochondria
Function: Catalyzes the urea cycle of ureotelic animals and remove excess ammonia from the cell
Interaction: Post-translational modification by SIRT5/Catalyze ammonia and bicarbonate to carbamoyl phosphate
Biology Area: Apoptosis/Tumor Suppressors/Cell Death, Cell Biology, Cell Proliferation and Viability, Mitochondrial Function, Neuroscience
Molecular Family: Enzymes and Regulators, Mitochondrial Markers
Antigen References: Nakagawa T, et al. 2009. Cell. 137(3):560-70. Diez-Fernandez C, et al. 2017. Expert Opin. Ther. Targets. 21:391-399. Li L, et al 2019. Nature. 567:253-256. Nitzahn M, et al. 2020. Mol Genet Metab. 131:289-298.
Gene ID: 1373
UniProt: View information about CPS1 on UniProt.org
Clone: W21062G
Regulatory Status: RUO
Other Names: Carbamoyl-Phosphate Synthetase I, Carbamoyl-Phosphate Synthase 1, Carbamoyl-Phosphate Synthase 1, Mitochondrial, Carbamoyl-Phosphate Synthase [Ammonia], Mitochondrial, CPSase I, CPSase 1
Isotype: Rat IgG2a, κ