Abcam, ab45956, Anti-CPS1 antibody

Size: 100µg
Rabbit Polyclonal CPS1 antibody. Suitable for IHC-P, WB, ICC/IF and reacts with Human samples. Cited in 22 publications.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:WB, IHC-P, ICC/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:The exact immunogen used to generate this antibody is proprietary information.

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7.4Preservative: 0.02% Sodium azideConstituents: PBS, 1% BSA, Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
CPS1 also known as carbamoyl-phosphate synthetase 1 is an important mitochondrial enzyme with a mass of around 165 kDa. It acts in the urea cycle by converting ammonia and bicarbonate into carbamoyl phosphate. CPS1 expression occurs in the liver and intestinal enterocytes which situates it in tissues responsible for metabolism and detoxification processes. This enzyme plays a critical role in the removal of excess nitrogen from the body.
Biological function summary
Carbamoyl-phosphate synthetase 1 functions as an initial step enzyme in the urea cycle which is a metabolic pathway for nitrogen disposal. It does not function alone as it forms part of multi-enzyme complexes to enhance efficiency in the cycle. These complexes facilitate the process of converting toxic ammonia into urea which the body can safely excrete. The proper function of CPS1 ensures that ammonia levels remain non-toxic.
Pathways
The urea cycle remains the chief pathway involving CPS1 collaborating with enzymes like ornithine transcarbamylase and argininosuccinate synthetase. This cycle intersects with the mitochondrial respiratory chain showcasing a relationship between CPS1 and cellular energy balance. Besides the urea cycle the enzyme also links to other metabolic functions like gluconeogenesis given its prerequisite for maintaining a stable nitrogen homeostasis.
CPS1 deficiencies can lead to urea cycle disorders like hyperammonemia characterized by elevated ammonia levels in blood. This may result in neurological damage if untreated. Another condition related to CPS-1 is pulmonary arterial hypertension where studies suggest mishandling of ammonia may alter blood pressure regulation. Genetic mutations affecting the CPS1 protein often underlie these conditions highlighting the importance of genetic screening in affected individuals.