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N-acetylglutamate synthase

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Plays a role in the regulation of ureagenesis by producing the essential cofactor N-acetylglutamate (NAG), thus modulating carbamoylphosphate synthase I (CPSI) activity.

Below are the list of possible N-acetylglutamate synthase products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

N-acetylglutamate synthase

 N-acetylglutamate synthase ELISA Kit
 N-acetylglutamate synthase Recombinant
 N-acetylglutamate synthase Antibody
Also known as N-acetylglutamate synthase, mitochondrial (Amino-acid acetyltransferase).
NAGS: Plays a role in the regulation of ureagenesis by producing variable amounts of N-acetylglutamate (NAG), thus modulating carbamoylphosphate synthase I (CPSI) activity. Defects in NAGS are the cause of N-acetylglutamate synthase deficiency (NAGSD). NAGSD is a rare autosomal recessively inherited metaboli
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c disorder leading to severe neonatal or late onset hyperammonemia without increased excretion of orotic acid. Clinical symptoms are somnolence, tachypnea, feeding difficulties, a severe neurologic presentation characterized by uncontrollable movements, developmental delay, visual impairment, failure to thrive and hyperammonemia precipitated by the introduction of high-protein diet or febrile illness. Belongs to the acetyltransferase family.

Protein type: Acetyltransferase; Amino Acid Metabolism - arginine and proline; EC 2.3.1.1; Mitochondrial

Cellular Component: mitochondrion

Molecular Function: amino-acid N-acetyltransferase activity

Biological Process: arginine biosynthetic process; glutamate metabolic process
 Nags ELISA Kit
 Nags Recombinant
 Nags Antibody
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