**Background of CPS1 Antibody**
The CPS1 (Carbamoyl Phosphate Synthetase 1) antibody is a crucial tool for studying the enzyme CPS1. a mitochondrial protein central to the urea cycle. CPS1 catalyzes the first and rate-limiting step of ammonia detoxification, converting ammonia into carbamoyl phosphate. This process is vital for nitrogen metabolism, particularly in the liver, where CPS1 is highly expressed. Dysregulation of CPS1 is linked to hyperammonemia and urea cycle disorders (UCDs), such as CPS1 deficiency, a rare autosomal recessive condition causing life-threatening ammonia accumulation.
CPS1 antibodies are widely used in research to detect protein expression, localization, and quantification via techniques like Western blotting, immunohistochemistry (IHC), and immunofluorescence (IF). These applications help investigate CPS1's role in metabolic diseases, liver dysfunction, and cancer, where altered CPS1 levels may correlate with tumor progression or therapeutic resistance. Additionally, studies explore CPS1's potential as a biomarker in mitochondrial disorders or metabolic syndromes.
Commercial CPS1 antibodies are typically raised against specific epitopes of human CPS1. with validation in various species and sample types. Researchers rely on these antibodies to advance understanding of urea cycle mechanics, develop diagnostic strategies, and explore targeted therapies for CPS1-related pathologies.