1MKO

A Fourth Quaternary Structure of Human Hemoglobin A at 2.18 A Resolution

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)
C	d1mkoc_	All alpha proteins	Globin-like	Globin-like	Globins	Hemoglobin, alpha-chain	(Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
A	d1mkoa_	All alpha proteins	Globin-like	Globin-like	Globins	Hemoglobin, alpha-chain	(Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d1mkob_	All alpha proteins	Globin-like	Globin-like	Globins	Hemoglobin, beta-chain	(Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
D	d1mkod_	All alpha proteins	Globin-like	Globin-like	Globins	Hemoglobin, beta-chain	(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)
C	SCOP2B Superfamily	Globin-like	8039836	3000554	SCOP2B (2022-06-29)
A	SCOP2B Superfamily	Globin-like	8039836	3000554	SCOP2B (2022-06-29)
B	SCOP2B Superfamily	Globin-like	8039838	3000554	SCOP2B (2022-06-29)
D	SCOP2B Superfamily	Globin-like	8039838	3000554	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)
C	Globin	e1mkoC1	A: alpha arrays	X: Globin-like (From Topology)	H: Globin-like (From Topology)	T: Globin-like	F: Globin	ECOD (1.6)
A	Globin	e1mkoA1	A: alpha arrays	X: Globin-like (From Topology)	H: Globin-like (From Topology)	T: Globin-like	F: Globin	ECOD (1.6)
B	Globin	e1mkoB1	A: alpha arrays	X: Globin-like (From Topology)	H: Globin-like (From Topology)	T: Globin-like	F: Globin	ECOD (1.6)
D	Globin	e1mkoD1	A: alpha arrays	X: Globin-like (From Topology)	H: Globin-like (From Topology)	T: Globin-like	F: Globin	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
C	1.10.490.10	Mainly Alpha	Orthogonal Bundle	Globin-like	Globins	CATH (4.3.0)
A	1.10.490.10	Mainly Alpha	Orthogonal Bundle	Globin-like	Globins	CATH (4.3.0)
B	1.10.490.10	Mainly Alpha	Orthogonal Bundle	Globin-like	Globins	CATH (4.3.0)
D	1.10.490.10	Mainly Alpha	Orthogonal Bundle	Globin-like	Globins	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, C	PF00042	Globin (Globin)	Globin	-	Domain
B, D	PF00042	Globin (Globin)	Globin	-	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, C	Hemoglobin alpha chain	oxygen binding binding small molecule binding oxygen carrier activity molecular carrier activity organic cyclic compound binding tetrapyrrole binding heme binding cation binding transition metal ion binding iron ion binding ion binding metal ion binding organic acid binding	catabolic process hydrogen peroxide metabolic process reactive oxygen species metabolic process metabolic process cellular process hydrogen peroxide catabolic process oxygen transport localization transport gas transport establishment of localization one-carbon compound transport carbon dioxide transport nitric oxide transport catabolic process hydrogen peroxide metabolic process reactive oxygen species metabolic process metabolic process cellular process hydrogen peroxide catabolic process oxygen transport localization transport gas transport establishment of localization one-carbon compound transport carbon dioxide transport nitric oxide transport nitrogen compound transport response to hydrogen peroxide response to oxidative stress response to stimulus response to stress response to chemical response to oxygen-containing compound response to reactive oxygen species cellular response to stimulus cellular detoxification cellular response to chemical stimulus detoxification cellular oxidant detoxification cellular response to toxic substance response to toxic substance	protein-containing complex cellular anatomical structure intracellular anatomical structure hemoglobin complex cytoplasm cytosol haptoglobin-hemoglobin complex membrane extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle extracellular region organelle protein-containing complex cellular anatomical structure intracellular anatomical structure hemoglobin complex cytoplasm cytosol haptoglobin-hemoglobin complex membrane extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle extracellular region organelle extracellular space vesicle extracellular membrane-bounded organelle blood microparticle intracellular vesicle membrane-enclosed lumen intracellular membrane-bounded organelle intracellular organelle lumen intracellular organelle cytoplasmic vesicle organelle lumen endocytic vesicle lumen endocytic vesicle
B, D	Hemoglobin beta chain	hemoglobin binding protein binding binding oxygen binding small molecule binding hemoglobin alpha binding oxygen carrier activity molecular carrier activity organic cyclic compound binding tetrapyrrole binding heme binding organic acid binding cation binding ion binding hemoglobin binding protein binding binding oxygen binding small molecule binding hemoglobin alpha binding oxygen carrier activity molecular carrier activity organic cyclic compound binding tetrapyrrole binding heme binding organic acid binding cation binding ion binding metal ion binding	regulation of biosynthetic process regulation of nitric oxide biosynthetic process regulation of biological process regulation of cellular process biological regulation positive regulation of biological process reactive nitrogen species metabolic process positive regulation of nitric oxide metabolic process nitric oxide metabolic process positive regulation of metabolic process nitric oxide biosynthetic process biosynthetic process positive regulation of biosynthetic process cellular process regulation of biosynthetic process regulation of nitric oxide biosynthetic process regulation of biological process regulation of cellular process biological regulation positive regulation of biological process reactive nitrogen species metabolic process positive regulation of nitric oxide metabolic process nitric oxide metabolic process positive regulation of metabolic process nitric oxide biosynthetic process biosynthetic process positive regulation of biosynthetic process cellular process metabolic process regulation of nitric oxide metabolic process positive regulation of nitric oxide biosynthetic process regulation of metabolic process positive regulation of cellular process catabolic process hydrogen peroxide metabolic process reactive oxygen species metabolic process hydrogen peroxide catabolic process multicellular organismal process system process circulatory system process regulation of blood pressure regulation of biological quality blood circulation regulation of body fluid levels wound healing response to stimulus homotypic cell-cell adhesion cell activation hemostasis platelet aggregation blood coagulation cell adhesion cell-cell adhesion response to stress coagulation response to wounding platelet activation oxygen transport localization transport gas transport establishment of localization one-carbon compound transport carbon dioxide transport renal system process renal absorption nitric oxide transport nitrogen compound transport regulation of tube diameter regulation of tube size vascular process in circulatory system blood vessel diameter maintenance regulation of anatomical structure size response to hydrogen peroxide response to oxidative stress response to chemical response to oxygen-containing compound response to reactive oxygen species cellular response to stimulus cellular detoxification cellular response to chemical stimulus detoxification cellular oxidant detoxification cellular response to toxic substance response to toxic substance	protein-containing complex cellular anatomical structure intracellular anatomical structure hemoglobin complex cytoplasm cytosol haptoglobin-hemoglobin complex extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle extracellular region organelle extracellular space protein-containing complex cellular anatomical structure intracellular anatomical structure hemoglobin complex cytoplasm cytosol haptoglobin-hemoglobin complex extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle extracellular region organelle extracellular space vesicle extracellular membrane-bounded organelle membrane-enclosed lumen intracellular vesicle ficolin-1-rich granule intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen intracellular organelle organelle lumen cytoplasmic vesicle ficolin-1-rich granule lumen secretory granule secretory vesicle blood microparticle tertiary granule lumen tertiary granule endocytic vesicle lumen endocytic vesicle

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, C	IPR002338	Hemoglobin, alpha-type	Family
A, C	IPR002339	Hemoglobin, pi	Family
A, C	IPR009050	Globin-like superfamily	Homologous Superfamily
A, C	IPR012292	Globin/Protoglobin	Homologous Superfamily
A, C	IPR000971	Globin	Domain
A, C	IPR050056	Hemoglobin and related oxygen transporters	Family
B, D	IPR009050	Globin-like superfamily	Homologous Superfamily
B, D	IPR012292	Globin/Protoglobin	Homologous Superfamily
B, D	IPR000971	Globin	Domain
B, D	IPR050056	Hemoglobin and related oxygen transporters	Family
B, D	IPR002337	Hemoglobin, beta-type	Family

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, C	Pharos: P69905	alpha thalassemia Heinz body anemia acquired polycythemia vera methemoglobinemia hemoglobin H disease polycythemia familial hemolytic anemia alpha thalassemia-intellectual disability syndrome type 1 sarcomatoid carcinoma carcinoma Mammary Neoplasms, Experimental type 2 diabetes mellitus cerebral infarction hemolytic anemia inborn carbohydrate metabolic disorder alpha thalassemia Heinz body anemia acquired polycythemia vera methemoglobinemia hemoglobin H disease polycythemia familial hemolytic anemia alpha thalassemia-intellectual disability syndrome type 1 sarcomatoid carcinoma carcinoma Mammary Neoplasms, Experimental type 2 diabetes mellitus cerebral infarction hemolytic anemia inborn carbohydrate metabolic disorder mammary neoplasms, animal thalassemia congenital anemia facioscapulohumeral muscular dystrophy familial polycythemia hydrops fetalis lung adenocarcinoma beta thalassemia sickle cell anemia Hb Bart's hydrops fetalis hemoglobin M disease maturity-onset diabetes of the young breast carcinoma undifferentiated carcinoma diabetes mellitus
B, D	Pharos: P68871	sickle cell anemia beta thalassemia dominant beta-thalassemia Heinz body anemia polycythemia methemoglobinemia alpha thalassemia echinococcosis acquired polycythemia vera malaria hemoglobin C disease Plasmodium falciparum malaria Cyanosis hemoglobin D disease inherited hemoglobinopathy sickle cell anemia beta thalassemia dominant beta-thalassemia Heinz body anemia polycythemia methemoglobinemia alpha thalassemia echinococcosis acquired polycythemia vera malaria hemoglobin C disease Plasmodium falciparum malaria Cyanosis hemoglobin D disease inherited hemoglobinopathy congenital dyserythropoietic anemia familial hemolytic anemia hemoglobin E disease beta-thalassemia HBB/LCRB hemoglobin E-beta-thalassemia syndrome juvenile dermatomyositis lung adenocarcinoma delta-beta-thalassemia lymphoplasmacytic lymphoma malignant ependymoma MALT lymphoma myopathy anemia gastric cancer pancreatic ductal adenocarcinoma atypical teratoid rhabdoid tumor pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia hemoglobin M disease beta-thalassemia major hereditary persistence of fetal hemoglobin cerebrovascular disorder sickle cell-hemoglobin E disease syndrome large cell medulloblastoma thalassemia minor lung cancer diabetes mellitus Duchenne muscular dystrophy malignant glioma medulloblastoma acute myeloid leukemia non-small cell lung carcinoma gastric carcinoma pancreatic intraductal papillary-mucinous carcinoma B-cell chronic lymphocytic leukemia periodontitis hemolytic anemia breast cancer hereditary persistence of fetal hemoglobin-beta-thalassemia syndrome sickle cell-hemoglobin c disease syndrome invasive ductal breast carcinoma thalassemia limb-girdle muscular dystrophy tuberculosis lung carcinoma malignant colon neoplasm ductal breast carcinoma in situ malignant pancreatic neoplasm exocrine pancreatic carcinoma familial polycythemia ovarian cancer atopic eczema head and neck cancer hemoglobin C-beta-thalassemia syndrome pancreatic intraductal papillary-mucinous neoplasm beta-thalassemia intermedia hepatocellular carcinoma breast carcinoma hypophosphatemic bone disease sickle cell-hemoglobin d disease syndrome congenital anemia

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.