4RLY

Crystal Structure of AnkB Ankyrin Repeats (R1-R9) in Complex with Nav1.2 Ankyrin Binding Domain

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	SCOP2 Family	Ankyrin repeat	8067327	4000366	SCOP2 (2022-06-29)
A	SCOP2 Superfamily	Ankyrin repeat	8067328	3000154	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	Ank_2	e4rlyA1	A: alpha superhelices	X: Repetitive alpha hairpins	H: Ankyrin repeat (From Topology)	T: Ankyrin repeat	F: Ank_2	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	1.25.40.20	Mainly Alpha	Alpha Horseshoe	Serine Threonine Protein Phosphatase 5, Tetratricopeptide repeat	Ankyrin repeat-containing domain	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A	PF06512	Sodium ion transport-associated (Na_trans_assoc)	Sodium ion transport-associated	-	Family
A	PF00023	Ankyrin repeat (Ank)	Ankyrin repeat	-	Repeat

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A	Nav1.2 - AnkB chimera	protein-macromolecule adaptor activity molecular adaptor activity ATPase binding protein binding binding enzyme binding cytoskeletal protein binding spectrin binding protein-containing complex binding transmembrane transporter binding kinase binding protein kinase binding structural constituent of cytoskeleton structural molecule activity protein-macromolecule adaptor activity molecular adaptor activity ATPase binding protein binding binding enzyme binding cytoskeletal protein binding spectrin binding protein-containing complex binding transmembrane transporter binding kinase binding protein kinase binding structural constituent of cytoskeleton structural molecule activity cytoskeletal anchor activity phosphorylation-dependent protein binding modification-dependent protein binding calcium channel activity monoatomic cation transmembrane transporter activity voltage-gated calcium channel activity gated channel activity monoatomic ion channel activity voltage-gated channel activity voltage-gated monoatomic ion channel activity voltage-gated monoatomic cation channel activity monoatomic cation channel activity passive transmembrane transporter activity calcium ion transmembrane transporter activity inorganic molecular entity transmembrane transporter activity channel activity inorganic cation transmembrane transporter activity monoatomic ion transmembrane transporter activity high voltage-gated calcium channel activity transporter activity metal ion transmembrane transporter activity transmembrane transporter activity voltage-gated sodium channel activity sodium channel activity sodium ion transmembrane transporter activity calmodulin binding	heart process multicellular organismal process system process regulation of biological process regulation of heart contraction regulation of system process biological regulation circulatory system process regulation of blood circulation regulation of multicellular organismal process regulation of biological quality heart contraction blood circulation regulation of heart rate heart process multicellular organismal process system process regulation of biological process regulation of heart contraction regulation of system process biological regulation circulatory system process regulation of blood circulation regulation of multicellular organismal process regulation of biological quality heart contraction blood circulation regulation of heart rate regulation of protein stability protein stabilization protein localization localization cellular localization cellular macromolecule localization macromolecule localization cellular process regulation of calcium ion transport regulation of localization transport regulation of transport regulation of monoatomic ion transport establishment of localization metal ion transport calcium ion transport monoatomic ion transport regulation of metal ion transport monoatomic cation transport response to organic substance response to stimulus response to chemical response to methylmercury protein localization to cell periphery protein localization to plasma membrane localization within membrane protein localization to membrane protein localization to T-tubule regulation of cellular process cellular biosynthetic process positive regulation of biological process positive regulation of cellular metabolic process positive regulation of macromolecule biosynthetic process positive regulation of metabolic process organic substance metabolic process positive regulation of biosynthetic process organic substance biosynthetic process positive regulation of macromolecule metabolic process positive regulation of cellular process regulation of biosynthetic process cellular metabolic process regulation of cellular metabolic process regulation of macromolecule metabolic process gene expression macromolecule biosynthetic process regulation of cellular biosynthetic process regulation of gene expression biosynthetic process positive regulation of gene expression regulation of macromolecule biosynthetic process metabolic process macromolecule metabolic process regulation of metabolic process positive regulation of cellular biosynthetic process cardiac muscle cell membrane repolarization membrane repolarization regulation of membrane repolarization ventricular cardiac muscle cell membrane repolarization regulation of cardiac muscle cell membrane repolarization regulation of ventricular cardiac muscle cell membrane repolarization regulation of membrane potential protein localization to organelle inorganic ion homeostasis intracellular calcium ion homeostasis homeostatic process calcium ion homeostasis cellular homeostasis chemical homeostasis intracellular monoatomic cation homeostasis monoatomic ion homeostasis intracellular chemical homeostasis monoatomic cation homeostasis intracellular monoatomic ion homeostasis SA node cell to atrial cardiac muscle cell signaling signaling cell-cell signaling involved in cardiac conduction SA node cell to atrial cardiac muscle cell communication cell communication regulation of cardiac muscle cell action potential cell communication involved in cardiac conduction regulation of action potential SA node cell action potential cell-cell signaling action potential cardiac conduction regulation of SA node cell action potential regulation of cell communication cardiac muscle cell action potential positive regulation of cation transmembrane transport regulation of potassium ion transport monoatomic ion transmembrane transport potassium ion transmembrane transport regulation of transmembrane transporter activity regulation of monoatomic ion transmembrane transport regulation of transporter activity monoatomic cation transmembrane transport positive regulation of molecular function positive regulation of monoatomic ion transport positive regulation of transmembrane transport positive regulation of monoatomic ion transmembrane transport regulation of potassium ion transmembrane transporter activity regulation of monoatomic ion transmembrane transporter activity inorganic ion transmembrane transport regulation of potassium ion transmembrane transport inorganic cation transmembrane transport transmembrane transport regulation of molecular function positive regulation of potassium ion transport regulation of transmembrane transport positive regulation of transport positive regulation of potassium ion transmembrane transport positive regulation of transporter activity positive regulation of ion transmembrane transporter activity potassium ion transport regulation of monoatomic cation transmembrane transport positive regulation of potassium ion transmembrane transporter activity positive regulation of calcium ion transport cellular response to stimulus negative regulation of biological process sarcoplasmic reticulum calcium ion transport release of sequestered calcium ion into cytosol by sarcoplasmic reticulum cardiac muscle contraction intracellular signal transduction intracellular signaling cassette signal transduction release of sequestered calcium ion into cytosol by endoplasmic reticulum regulation of release of sequestered calcium ion into cytosol regulation of cardiac muscle contraction muscle contraction maintenance of location release of sequestered calcium ion into cytosol regulation of striated muscle contraction calcium-mediated signaling regulation of muscle contraction negative regulation of sequestering of calcium ion regulation of cardiac muscle contraction by regulation of the release of sequestered calcium ion maintenance of location in cell striated muscle contraction regulation of calcium ion transmembrane transport negative regulation of cellular process regulation of sequestering of calcium ion calcium ion transmembrane import into cytosol regulation of cardiac muscle contraction by calcium ion signaling sequestering of calcium ion muscle system process regulation of muscle system process calcium ion transmembrane transport regulation of release of sequestered calcium ion into cytosol by sarcoplasmic reticulum membrane organization developmental process cellular developmental process cell development muscle cell differentiation cellular component organization muscle structure development T-tubule organization muscle cell development cell differentiation plasma membrane organization anatomical structure development cellular component organization or biogenesis endomembrane system organization actin filament-based process cardiac muscle cell contraction regulation of actin filament-based movement regulation of atrial cardiac muscle cell action potential cardiac muscle cell action potential involved in contraction regulation of actin filament-based process regulation of cardiac conduction atrial cardiac muscle cell to AV node cell signaling atrial cardiac muscle cell to AV node cell communication atrial cardiac muscle cell action potential actin filament-based movement regulation of cardiac muscle cell contraction actin-mediated cell contraction regulation of heart rate by cardiac conduction ventricular cardiac muscle cell action potential positive regulation of cation channel activity regulation of cation channel activity protein localization to cell surface import into cell endocytosis vesicle-mediated transport establishment of protein localization nitrogen compound transport organic substance transport protein transport regulation of calcium ion transmembrane transporter activity membrane depolarization during cardiac muscle cell action potential membrane depolarization during action potential membrane depolarization membrane depolarization during SA node cell action potential positive regulation of calcium ion transmembrane transporter activity positive regulation of calcium ion transmembrane transport heart development animal organ development cardiac atrium development circulatory system development multicellular organism development cardiac septum development cardiac chamber development atrial septum development system development protein localization to endoplasmic reticulum cellular component assembly involved in morphogenesis myelin assembly cellular component biogenesis cellular anatomical entity morphogenesis cellular component assembly cell junction assembly axon ensheathment ensheathment of neurons cell-cell junction assembly cell-cell junction organization nervous system development myelination anatomical structure morphogenesis cell junction organization anatomical structure formation involved in morphogenesis paranodal junction assembly protein localization to M-band response to decreased oxygen levels cellular response to oxygen levels cellular response to hypoxia cellular response to decreased oxygen levels cellular response to chemical stimulus cellular response to stress response to hypoxia response to stress response to oxygen levels response to abiotic stimulus cellular response to chemical stress cellular response to environmental stimulus intrinsic apoptotic signaling pathway response to osmotic stress cellular response to osmotic stress cell death apoptotic signaling pathway intrinsic apoptotic signaling pathway in response to osmotic stress cellular response to abiotic stimulus apoptotic process programmed cell death behavior learning or memory nervous system process cognition memory import across plasma membrane inorganic cation import across plasma membrane calcium ion import across plasma membrane inorganic ion import across plasma membrane calcium ion import calcium ion import into cytosol transmission of nerve impulse neuronal action potential determination of adult lifespan neuron apoptotic process sodium ion transport head development forebrain development brain development dentate gyrus development pallium development central nervous system development telencephalon development hippocampus development limbic system development nerve development	cell periphery postsynapse postsynaptic membrane synapse cell junction membrane plasma membrane plasma membrane region cellular anatomical entity synaptic membrane sarcolemma T-tubule M band intracellular anatomical structure cell periphery postsynapse postsynaptic membrane synapse cell junction membrane plasma membrane plasma membrane region cellular anatomical entity synaptic membrane sarcolemma T-tubule M band intracellular anatomical structure sarcomere intracellular organelle organelle intracellular non-membrane-bounded organelle contractile muscle fiber A band cytoplasm supramolecular fiber myofibril supramolecular polymer supramolecular complex non-membrane-bounded organelle Z disc I band cytosol costamere basal part of cell basal plasma membrane basolateral plasma membrane cell projection neuron projection plasma membrane bounded cell projection cytoskeleton membrane-bounded organelle endosome intracellular vesicle intracellular membrane-bounded organelle recycling endosome endomembrane system cytoplasmic vesicle vesicle cell-cell junction intercalated disc anchoring junction cell-cell contact zone mitochondrion apical part of cell apical plasma membrane lytic vacuole vacuole lysosome early endosome axon paranode region of axon main axon node of Ranvier presynapse presynaptic membrane glutamatergic synapse protein-containing complex sodium channel complex voltage-gated sodium channel complex plasma membrane protein complex membrane protein complex cation channel complex transporter complex transmembrane transporter complex monoatomic ion channel complex voltage-gated calcium channel complex calcium channel complex

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR040745	Ankyrin, UPA domain	Domain
A	IPR000906	ZU5 domain	Domain
A	IPR002110	Ankyrin repeat	Repeat
A	IPR000488	Death domain	Domain
A	IPR036770	Ankyrin repeat-containing domain superfamily	Homologous Superfamily
A	IPR011029	Death-like domain superfamily	Homologous Superfamily
A	IPR024583	Voltage-gated Na+ ion channel, cytoplasmic domain	Domain
A	IPR001696	Voltage gated sodium channel, alpha subunit	Family
A	IPR000048	IQ motif, EF-hand binding site	Binding Site
A	IPR010526	Sodium ion transport-associated domain	Domain
A	IPR005821	Ion transport domain	Domain
A	IPR044564	Voltage-gated sodium channel alpha subunit, inactivation gate	Domain
A	IPR043203	Voltage-gated cation channel calcium and sodium	Family
A	IPR027359	Voltage-dependent channel domain superfamily	Homologous Superfamily

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	Pharos: Q99250	cardiac arrhythmia, ankyrin-B-related long QT syndrome cerebrovascular disorder Neurodevelopmental Disorders familial long QT syndrome invasive ductal breast carcinoma long QT syndrome 1 lung carcinoma malignant glioma atopic eczema medulloblastoma autosomal dominant cardiac arrhythmia (Kuhn) non-small cell lung carcinoma Brugada syndrome pancreatic intraductal papillary-mucinous carcinoma cardiac arrhythmia, ankyrin-B-related long QT syndrome cerebrovascular disorder Neurodevelopmental Disorders familial long QT syndrome invasive ductal breast carcinoma long QT syndrome 1 lung carcinoma malignant glioma atopic eczema medulloblastoma autosomal dominant cardiac arrhythmia (Kuhn) non-small cell lung carcinoma Brugada syndrome pancreatic intraductal papillary-mucinous carcinoma Pick disease catecholaminergic polymorphic ventricular tachycardia primitive neuroectodermal tumor Down syndrome glioblastoma large cell medulloblastoma malignant colon neoplasm acute myeloid leukemia malignant mesothelioma atypical teratoid rhabdoid tumor nephrosclerosis B-cell chronic lymphocytic leukemia oligodendroglioma pancreatic intraductal papillary-mucinous neoplasm pilocytic astrocytoma catecholaminergic polymorphic ventricular tachycardia 1 psoriasis colorectal cancer ductal breast carcinoma in situ hereditary spastic paraplegia lung cancer malignant ependymoma adrenal cortex carcinoma autism spectrum disorder breast carcinoma ovarian cancer cardiomyopathy pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia pituitary cancer dermatomyositis epilepsy developmental and epileptic encephalopathy, 11 West syndrome seizures, benign familial infantile, 3 seizures, benign familial neonatal, 3 Seizures, Febrile generalized epilepsy with febrile seizures plus developmental and epileptic encephalopathy, 6 medulloblastoma epilepsy with generalized tonic-clonic seizures oligodendroglioma Pediculus humanus capitis infestation tinea pedis urethritis glioblastoma premature ejaculation primitive neuroectodermal tumor psoriasis attention deficit hyperactivity disorder, inattentive type autism spectrum disorder scabies benign familial neonatal epilepsy breast cancer malignant glioma developmental and epileptic encephalopathy Dravet syndrome Sjogren syndrome neuralgia status epilepticus osteosarcoma tinea corporis pilocytic astrocytoma hemorrhoid large cell medulloblastoma astrocytic tumor Pthirus pubis infestation atypical teratoid rhabdoid tumor lung cancer scrapie bipolar disorder malaria malignant mesothelioma subependymal giant cell astrocytoma partial epilepsy postherpetic neuralgia intellectual disability Lennox-Gastaut syndrome atrial fibrillation benign familial infantile epilepsy lung carcinoma malignant ependymoma

RCSB PDB Core Operations are funded by the National Science Foundation (DBI-1832184), 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.