4K30

Structure of the N-acetyltransferase domain of human N-acetylglutamate synthase

External Resource: Annotation

Domain Annotation: SCOP2 Classification
Domain Annotation: ECOD Classification
Domain Annotation: CATH
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

Chains	Type	Family Name	Domain Identifier	Family Identifier	Provenance Source (Version)
A	SCOP2B Superfamily	Acyl-CoA N-acyltransferases (Nat)	8101144	3000403	SCOP2B (2022-06-29)
B	SCOP2B Superfamily	Acyl-CoA N-acyltransferases (Nat)	8101144	3000403	SCOP2B (2022-06-29)
C [auth X]	SCOP2 Family	N-acetylglutamate synthase NAT domain-like	8046429	4005074	SCOP2 (2022-06-29)
C [auth X]	SCOP2 Superfamily	Acyl-CoA N-acyltransferases (Nat)	8101144	3000403	SCOP2 (2022-06-29)
D [auth Y]	SCOP2B Superfamily	Acyl-CoA N-acyltransferases (Nat)	8101144	3000403	SCOP2B (2022-06-29)

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
A	PF04768	e4k30A1	A: a+b three layers	X: Nat/Ivy	H: Acyl-CoA N-acyltransferases (Nat) (From Topology)	T: Acyl-CoA N-acyltransferases (Nat)	F: PF04768	ECOD (1.6)
B	PF04768	e4k30B1	A: a+b three layers	X: Nat/Ivy	H: Acyl-CoA N-acyltransferases (Nat) (From Topology)	T: Acyl-CoA N-acyltransferases (Nat)	F: PF04768	ECOD (1.6)
C [auth X]	PF04768	e4k30X1	A: a+b three layers	X: Nat/Ivy	H: Acyl-CoA N-acyltransferases (Nat) (From Topology)	T: Acyl-CoA N-acyltransferases (Nat)	F: PF04768	ECOD (1.6)
D [auth Y]	PF04768	e4k30Y1	A: a+b three layers	X: Nat/Ivy	H: Acyl-CoA N-acyltransferases (Nat) (From Topology)	T: Acyl-CoA N-acyltransferases (Nat)	F: PF04768	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	3.40.630.30	Alpha Beta	3-Layer(aba) Sandwich	Aminopeptidase	Gcn5-related N-acetyltransferase (GNAT)	CATH (4.3.0)
B	3.40.630.30	Alpha Beta	3-Layer(aba) Sandwich	Aminopeptidase	Gcn5-related N-acetyltransferase (GNAT)	CATH (4.3.0)
C [auth X]	3.40.630.30	Alpha Beta	3-Layer(aba) Sandwich	Aminopeptidase	Gcn5-related N-acetyltransferase (GNAT)	CATH (4.3.0)
D [auth Y]	3.40.630.30	Alpha Beta	3-Layer(aba) Sandwich	Aminopeptidase	Gcn5-related N-acetyltransferase (GNAT)	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, B, C [auth X], D [auth Y]	PF04768	NAT, N-acetyltransferase, of N-acetylglutamate synthase (NAT)	NAT, N-acetyltransferase, of N-acetylglutamate synthase	-	Family

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, B, C [auth X], D [auth Y]	N-acetylglutamate synthase, mitochondrial	acyltransferase activity methione N-acyltransferase activity acyltransferase activity, transferring groups other than amino-acyl groups transferase activity catalytic activity acetyltransferase activity N-acetyltransferase activity acetyl-CoA:L-glutamate N-acetyltransferase activity N-acyltransferase activity	oxoacid metabolic process glutamine family amino acid biosynthetic process cellular biosynthetic process organonitrogen compound biosynthetic process amino acid metabolic process organic acid metabolic process organic acid biosynthetic process arginine biosynthetic process alpha-amino acid metabolic process carboxylic acid biosynthetic process glutamine family amino acid metabolic process arginine metabolic process organic substance metabolic process organic substance biosynthetic process oxoacid metabolic process glutamine family amino acid biosynthetic process cellular biosynthetic process organonitrogen compound biosynthetic process amino acid metabolic process organic acid metabolic process organic acid biosynthetic process arginine biosynthetic process alpha-amino acid metabolic process carboxylic acid biosynthetic process glutamine family amino acid metabolic process arginine metabolic process organic substance metabolic process organic substance biosynthetic process small molecule metabolic process organonitrogen compound metabolic process small molecule biosynthetic process cellular metabolic process carboxylic acid metabolic process primary metabolic process L-amino acid biosynthetic process L-amino acid metabolic process proteinogenic amino acid metabolic process amino acid biosynthetic process biosynthetic process proteinogenic amino acid biosynthetic process metabolic process cellular process alpha-amino acid biosynthetic process dicarboxylic acid metabolic process glutamate metabolic process amide biosynthetic process urea metabolic process nitrogen cycle metabolic process urea cycle amide metabolic process	membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen organelle lumen

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, B, C [auth X], D [auth Y]	IPR001048	Aspartate/glutamate/uridylate kinase	Domain
A, B, C [auth X], D [auth Y]	IPR006855	Vertebrate-like NAGS Gcn5-related N-acetyltransferase (GNAT) domain	Domain
A, B, C [auth X], D [auth Y]	IPR000182	GNAT domain	Domain
A, B, C [auth X], D [auth Y]	IPR016181	Acyl-CoA N-acyltransferase	Homologous Superfamily
A, B, C [auth X], D [auth Y]	IPR011243	N-acetylglutamate synthase, animal	Family
A, B, C [auth X], D [auth Y]	IPR036393	Acetylglutamate kinase-like superfamily	Homologous Superfamily

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, B, C [auth X], D [auth Y]	Pharos: Q8N159	hyperammonemia due to N-acetylglutamate synthase deficiency Hyperammonemia argininosuccinic aciduria urea cycle disorder intellectual disability

RCSB PDB Core Operations are funded by the U.S. National Science Foundation (DBI-2321666), the US Department of Energy (DE-SC0019749), and the National Cancer Institute, National Institute of Allergy and Infectious Diseases, and National Institute of General Medical Sciences of the National Institutes of Health under grant R01GM133198.