1HH8 | pdb_00001hh8

The active N-terminal region of p67phox: Structure at 1.8 Angstrom resolution and biochemical characterizations of the A128V mutant implicated in chronic granulomatous disease

External Resource: Annotation

Domain Annotation: SCOP/SCOPe Classification
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: SCOP/SCOPe Classification SCOP-e Database Homepage

Chains	Domain Info	Class	Fold	Superfamily	Family	Domain	Species	Provenance Source (Version)
A	d1hh8a_	All alpha proteins	alpha-alpha superhelix	TPR-like	Tetratricopeptide repeat (TPR)	Neutrophil cytosolic factor 2 (NCF-2, p67-phox)	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

Chains	Type	Family Name	Domain Identifier	Family Identifier	Provenance Source (Version)
A	SCOP2 Family	Tetratricopeptide repeat (TPR)	8020781	4001344	SCOP2 (2022-06-29)
A	SCOP2 Superfamily	TPR-like	8033161	3001345	SCOP2 (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	TPR_11_2	e1hh8A1	A: alpha superhelices	X: Repetitive alpha hairpins	H: ARM repeat (From Topology)	T: ARM repeat	F: TPR_11_2	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	1.25.40.10	Mainly Alpha	Alpha Horseshoe	Serine Threonine Protein Phosphatase 5, Tetratricopeptide repeat	Tetratricopeptide repeat domain	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A	PF13181	Tetratricopeptide repeat (TPR_8)	Tetratricopeptide repeat	-	Repeat

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A	NEUTROPHIL CYTOSOL FACTOR 2	small GTPase binding protein binding binding enzyme binding GTPase binding electron transfer activity superoxide-generating NADPH oxidase activator activity enzyme regulator activity enzyme activator activity molecular function activator activity molecular function regulator activity oxidoreductase activity superoxide-generating NAD(P)H oxidase activity oxidoreductase activity, acting on NAD(P)H small GTPase binding protein binding binding enzyme binding GTPase binding electron transfer activity superoxide-generating NADPH oxidase activator activity enzyme regulator activity enzyme activator activity molecular function activator activity molecular function regulator activity oxidoreductase activity superoxide-generating NAD(P)H oxidase activity oxidoreductase activity, acting on NAD(P)H catalytic activity oxidoreductase activity, acting on NAD(P)H, oxygen as acceptor	superoxide metabolic process superoxide anion generation reactive oxygen species metabolic process metabolic process cellular process localization import into cell transport phagocytosis endocytosis establishment of localization vesicle-mediated transport biological process involved in interspecies interaction between organisms response to stimulus superoxide metabolic process superoxide anion generation reactive oxygen species metabolic process metabolic process cellular process localization import into cell transport phagocytosis endocytosis establishment of localization vesicle-mediated transport biological process involved in interspecies interaction between organisms response to stimulus immune response response to other organism defense response defense response to symbiont response to external stimulus response to biotic stimulus immune system process defense response to other organism response to stress innate immune response response to external biotic stimulus cellular defense response respiratory burst	cellular anatomical structure membrane membrane-bounded organelle phagolysosome intracellular anatomical structure lytic vacuole intracellular vesicle vacuole phagocytic vesicle intracellular membrane-bounded organelle intracellular organelle cytoplasmic vesicle organelle secondary lysosome cellular anatomical structure membrane membrane-bounded organelle phagolysosome intracellular anatomical structure lytic vacuole intracellular vesicle vacuole phagocytic vesicle intracellular membrane-bounded organelle intracellular organelle cytoplasmic vesicle organelle secondary lysosome cytoplasm vesicle endocytic vesicle lysosome cytosol cell periphery plasma membrane protein-containing complex catalytic complex plasma membrane protein complex NADPH oxidase complex membrane protein complex oxidoreductase complex acrosomal vesicle endomembrane system secretory granule secretory vesicle

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR051864	NADPH Oxidase Activator	Family
A	IPR011990	Tetratricopeptide-like helical domain superfamily	Homologous Superfamily
A	IPR000270	PB1 domain	Domain
A	IPR036028	SH3-like domain superfamily	Homologous Superfamily
A	IPR035546	Neutrophil cytosol factor 2, SH3 domain 1	Domain
A	IPR019734	Tetratricopeptide repeat	Repeat
A	IPR034889	Neutrophil cytosol factor 2, SH3 domain	Domain
A	IPR053793	PB1-like domain	Domain
A	IPR001452	SH3 domain	Domain
A	IPR034885	Neutrophil cytosol factor 2, PB1 domain	Domain

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	Pharos: P19878	chronic granulomatous disease granulomatous disease, chronic, autosomal recessive, cytochrome b-positive, type 2 rheumatoid arthritis systemic lupus erythematosus osteosarcoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia breast cancer cystic fibrosis invasive ductal breast carcinoma lung carcinoma malignant glioma myositis ovarian cancer phagocyte bactericidal dysfunction systemic sclerosis chronic granulomatous disease granulomatous disease, chronic, autosomal recessive, cytochrome b-positive, type 2 rheumatoid arthritis systemic lupus erythematosus osteosarcoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia breast cancer cystic fibrosis invasive ductal breast carcinoma lung carcinoma malignant glioma myositis ovarian cancer phagocyte bactericidal dysfunction systemic sclerosis celiac disease diabetes mellitus IgA glomerulonephritis lung adenocarcinoma lupus erythematosus malignant pancreatic neoplasm non-small cell lung carcinoma pancreatic intraductal papillary-mucinous carcinoma ulcerative colitis glioblastoma interstitial cystitis lung cancer malignant ependymoma MALT lymphoma

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 R01GM157729.