Anti-NAGS Rabbit Polyclonal Antibody for WB, ELISA - Q8N159

Core Product Specifications and Parameters

Biological Background: NAGS Function and Localization

• NAGS encodes N-acetylglutamate synthase, an amino-acid acetyltransferase that produces N-acetylglutamate (NAG), an essential allosteric cofactor for carbamoylphosphate synthase I (CPS1), the rate-limiting enzyme of the urea cycle.
• By generating NAG, NAGS critically regulates ureagenesis, ammonia detoxification, and nitrogen homeostasis in hepatocytes.
• The enzyme is localized in the mitochondrial matrix, where the urea cycle operates.
• NAGS is highly expressed in adult liver, kidney, and small intestine; weaker expression is detected in fetal tissues, lung, pancreas, placenta, heart, and brain.
• Loss-of-function mutations in NAGS cause autosomal recessive NAGS deficiency, a rare urea cycle disorder characterized by neonatal or late-onset hyperammonemia.
• The protein contains an N-terminal mitochondrial transit peptide and belongs to the acyltransferase family; its three-dimensional structure has been solved, offering insights into substrate binding and catalytic mechanism.

Experimental Guidance and Technical Tips

• The immunogen spans amino acids 295–534, corresponding to the C-terminal domain of human NAGS; thus, the antibody may detect full-length and certain truncated forms.
• For WB, a band around 58 kDa is expected. Mitochondrial fractions or liver/kidney lysates are recommended as positive controls.
• ELISA performance should be validated using the recombinant immunogen as a standard to confirm binding specificity.
• Cross-reactivity with mouse and rat NAGS is reported; however, sequence alignment of the immunogen region across species is advised to ensure epitope conservation.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio serves as a comprehensive IVD raw-material sourcing partner for diagnostic manufacturers, laboratories, and research institutes. This anti-NAGS polyclonal antibody supports urea cycle and metabolic disease research. Our supply capabilities encompass validated antibody pairs, optimized polyclonal and monoclonal antibodies, and bulk ancillary reagents to streamline your assay development.