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The antibody against NAGS was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 295-534 of human NAGS (NP_694551.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
The antibody against NAGS was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 295-534 of human NAGS (NP_694551.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
| Cat.No | ADA-09257A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | NAGS |
| Target Synonyms | AGAS; ARGA; NAGS | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.01% thimerosal, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | 293T, K-562, Mouse liver, Mouse small intestine, Mouse testis, Rat liver | Application | ELISA, WB |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 295-534 of human NAGS (NP_694551.1). | Target Species | Human |
|---|---|---|---|
| Uniprot ID | Q8N159 | Immunogen Sequence |
Uniprot Id
Q8N159
Target Species
Human
Target Name
NAGS
Target Full Name
N-acetylglutamate synthase, mitochondrial
Target Function
Plays a role in the regulation of ureagenesis by producing the essential cofactor N-acetylglutamate (NAG), thus modulating carbamoylphosphate synthase I (CPS1) activity.
Target Involvement
N-acetylglutamate synthase deficiency (NAGSD)
Target Subcellular Location
Mitochondrion matrix.
Target Protein Families
Acetyltransferase family
Target Tissue Specificity
Highly expressed in the adult liver, kidney and small intestine. Weakly expressed in the fetal liver, lung, pancreas, placenta, heart and brain tissue.
Target Synonyms
NAGS; N-acetylglutamate synthase; mitochondrial; EC 2.3.1.1; Amino-acid acetyltransferase) [Cleaved into: N-acetylglutamate synthase long form; N-acetylglutamate synthase short form; N-acetylglutamate synthase conserved domain form]
Target Background
The N-acetylglutamate synthase gene encodes a mitochondrial enzyme that catalyzes the formation of N-acetylglutamate (NAG) from glutamate and acetyl coenzyme-A. NAG is a cofactor of carbamyl phosphate synthetase I (CPSI), the first enzyme of the urea cycle in mammals. This gene may regulate ureagenesis by altering NAG availability and, thereby, CPSI activity. Deficiencies in N-acetylglutamate synthase have been associated with hyperammonemia.
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