Description
Key facts
Cell type:HeLa,
Species or organism:Human,
Tissue:Cervix,
Form:LiquidSee storage information,
Knockout validation:Sanger Sequencing,Western blot,
Mutation description:Knockout achieved by using CRISPR/Cas9, 1 bp insertion in exon 15 and Insertion of the selection cassette in exon 15,
Disease:Adenocarcinoma
Product details:
We will provide viable cells that proliferate on revival.
This product is subject to limited use licenses from The Broad Institute, ERS Genomics Limited and Sigma-Aldrich Co. LLC, and is developed with patented technology. For full details of the licenses and patents please refer to our
limited use license
patent pages
Properties and Storage Information:
Gene name-CPS1, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, Western blot, Shipped at conditions-Dry Ice, Appropriate short-term storage conditions--196°C, Appropriate long-term storage conditions--196°C
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.
