Metabolism Antibodies 1

Anti-NAGS Antibody (CAB12086)

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SKU:
CAB12086
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism

Description

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Antibody Name:Anti-NAGS Antibody
Antibody SKU:CAB12086
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 295-534 of human NAGS (NP_694551.1).
Application:WB
Recommended Dilution:WB 1:500 - 1:1000
Reactivity:Human, Mouse, Rat
Positive Samples:293T, K-562, Mouse liver, Mouse small intestine, Mouse testis, Rat liver
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 295-534 of human NAGS (NP_694551.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:NTGG LRDS SHKV LSNV NLPA DLDL VCNA EWVS TKER QQMR LIVD VLSR LPHH SSAV ITAA STLL TELF SNKG SGTL FKNA ERML RVRS LDKL DQGR LVDL VNAS FGKK LRDD YLAS LRPR LHSI YVSE GYNA AAIL TMEP VLGG TPYL DKFV VSSS RQGQ GSGQ MLWE CLRR DLQT LFWR SRVT NPIN PWYF KHSD GSFS NKQW IFFW FGLA DIRD SYEL VNHA KGLP DSFH KPAS DPGS
Gene ID:162417
Uniprot:Q8N159
Cellular Location:Mitochondrion matrix
Calculated MW:58kDa
Observed MW:58kDa
Synonyms:NAGS, AGAS, ARGA
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.
UniProt Protein Function: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 metabolic 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.
UniProt Protein Details:

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

Chromosomal Location of Human Ortholog: 17q21.31

Cellular Component: mitochondrial matrix

Molecular Function:amino-acid N-acetyltransferase activity

Biological Process: glutamate metabolic process; arginine biosynthetic process; urea cycle

Disease: N-acetylglutamate Synthase Deficiency

NCBI Summary: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. [provided by RefSeq, Jul 2008]
UniProt Code:Q8N159
NCBI GenInfo Identifier:74714699
NCBI Gene ID:162417
NCBI Accession:Q8N159.1
UniProt Related Accession:Q8N159
Molecular Weight:
NCBI Full Name:N-acetylglutamate synthase, mitochondrial
NCBI Synonym Full Names:N-acetylglutamate synthase
NCBI Official Symbol:NAGS  
NCBI Official Synonym Symbols:AGAS; ARGA  
NCBI Protein Information:N-acetylglutamate synthase, mitochondrial
UniProt Protein Name:N-acetylglutamate synthase, mitochondrial
UniProt Synonym Protein Names:Amino-acid acetyltransferaseCleaved into the following 3 chains:N-acetylglutamate synthase long form; N-acetylglutamate synthase short form; N-acetylglutamate synthase conserved domain form
Protein Family:N-acetylglutamate synthase
UniProt Gene Name:NAGS  
UniProt Entry Name:NAGS_HUMAN
Anti-NAGS Antibody (CAB12086)Western blot analysis of extracts of various cell lines, using NAGS antibody (CAB12086) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
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