1YA5

Crystal structure of the titin domains z1z2 in complex with telethonin

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	d1ya5a1	All beta proteins	Immunoglobulin-like beta-sandwich	Immunoglobulin	automated matches	automated matches	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
A	d1ya5a3	Artifacts	Tags	Tags	Tags	C-terminal Tags	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d1ya5b1	All beta proteins	Immunoglobulin-like beta-sandwich	Immunoglobulin	automated matches	automated matches	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d1ya5b3	Artifacts	Tags	Tags	Tags	C-terminal Tags	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)
C [auth T]	SCOP2 Family	Telethonin-like	8044933	4004210	SCOP2 (2022-06-29)
C [auth T]	SCOP2 Superfamily	Telethonin-like	8053807	3002230	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	PF07679	e1ya5A1	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: PF07679	ECOD (1.6)
B	PF07679	e1ya5B1	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: PF07679	ECOD (1.6)
C [auth T]	PF09470	e1ya5T1	A: beta meanders	X: Telethonin (From Topology)	H: Telethonin (From Topology)	T: Telethonin	F: PF09470	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	2.60.40.10	Mainly Beta	Sandwich	Immunoglobulin-like	Immunoglobulins	CATH (4.3.0)
B	2.60.40.10	Mainly Beta	Sandwich	Immunoglobulin-like	Immunoglobulins	CATH (4.3.0)
C [auth T]	2.20.160.10	Mainly Beta	Single Sheet	titin filament fold	titin domain like	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, B	PF07679	Immunoglobulin I-set domain (I-set)	Immunoglobulin I-set domain	-	Domain
C [auth T]	PF09470	Telethonin protein (Telethonin)	Telethonin protein	Telethonin is a 167-residue protein which complexes with the large muscle protein, titin. The very N-terminus of titin, composed of two immunoglobulin-like (Ig) domains, referred to as Z1 and Z2, interacts with the N-terminal region (residues 1-53) o ...	Telethonin is a 167-residue protein which complexes with the large muscle protein, titin. The very N-terminus of titin, composed of two immunoglobulin-like (Ig) domains, referred to as Z1 and Z2, interacts with the N-terminal region (residues 1-53) of telethonin, mediating the antiparallel assembly of two Z1Z2 domains. The C-terminus of the telethonin appears to induce dimerisation of this 2:1 titin/telethonin structure which thus forms a complex necessary for myofibril assembly and maintenance of the intact Z-disk of skeletal and cardiac muscles [1].	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, B	N2B-TITIN ISOFORM	cytoskeletal protein binding actin binding protein-containing complex binding protein binding binding actin filament binding structural molecule activity structural constituent of muscle alpha-actinin binding actinin binding muscle alpha-actinin binding protein tyrosine kinase activity catalytic activity, acting on a protein kinase activity cytoskeletal protein binding actin binding protein-containing complex binding protein binding binding actin filament binding structural molecule activity structural constituent of muscle alpha-actinin binding actinin binding muscle alpha-actinin binding protein tyrosine kinase activity catalytic activity, acting on a protein kinase activity protein kinase activity transferase activity catalytic activity phosphotransferase activity, alcohol group as acceptor transferase activity, transferring phosphorus-containing groups adenyl ribonucleotide binding nucleotide binding purine ribonucleotide binding purine ribonucleoside triphosphate binding nucleoside phosphate binding ion binding purine nucleotide binding organic cyclic compound binding anion binding adenyl nucleotide binding ATP binding heterocyclic compound binding carbohydrate derivative binding ribonucleotide binding small molecule binding calmodulin binding identical protein binding cation binding calcium ion binding metal ion binding enzyme binding protease binding structural molecule activity conferring elasticity protein serine kinase activity kinase binding protein kinase binding protein serine/threonine kinase activity telethonin binding	multicellular organismal process system process muscle contraction muscle system process striated muscle contraction response to stimulus response to metal ion response to chemical response to calcium ion response to inorganic substance regulation of cellular process cellular biosynthetic process positive regulation of biological process positive regulation of cellular metabolic process multicellular organismal process system process muscle contraction muscle system process striated muscle contraction response to stimulus response to metal ion response to chemical response to calcium ion response to inorganic substance 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 regulation of biological process cellular metabolic process biological regulation 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 cellular process macromolecule metabolic process regulation of metabolic process positive regulation of cellular biosynthetic process cellular developmental process cellular component assembly involved in morphogenesis non-membrane-bounded organelle assembly skeletal myofibril assembly cell development muscle cell differentiation cellular component organization cellular component biogenesis muscle structure development striated muscle cell development cellular anatomical entity morphogenesis cellular component assembly muscle cell development cell differentiation actin filament-based process anatomical structure development cellular component organization or biogenesis developmental process skeletal muscle thin filament assembly actin cytoskeleton organization actomyosin structure organization organelle assembly actin filament organization anatomical structure morphogenesis organelle organization myofibril assembly anatomical structure formation involved in morphogenesis striated muscle cell differentiation supramolecular fiber organization cytoskeleton organization mitotic nuclear division mitotic chromosome condensation organelle fission chromosome organization sister chromatid segregation mitotic cell cycle nuclear division mitotic cell cycle process chromosome condensation mitotic sister chromatid segregation nuclear chromosome segregation cell cycle chromosome segregation cell cycle process phosphorylation phosphate-containing compound metabolic process phosphorus metabolic process detection of external stimulus detection of stimulus detection of mechanical stimulus detection of muscle stretch response to mechanical stimulus response to external stimulus response to abiotic stimulus detection of abiotic stimulus response to muscle stretch regulation of protein modification process regulation of catalytic activity regulation of protein phosphorylation regulation of transferase activity regulation of primary metabolic process regulation of phosphate metabolic process protein metabolic process protein modification process organonitrogen compound metabolic process protein phosphorylation regulation of protein metabolic process primary metabolic process regulation of protein kinase activity regulation of molecular function regulation of kinase activity macromolecule modification regulation of phosphorylation regulation of phosphorus metabolic process myosin filament assembly protein-containing complex assembly skeletal muscle myosin thick filament assembly protein-containing complex organization myosin filament organization striated muscle myosin thick filament assembly heart development cardiac muscle tissue development heart morphogenesis animal organ development tissue development muscle tissue morphogenesis striated muscle tissue development circulatory system development multicellular organism development tissue morphogenesis cardiac muscle tissue morphogenesis animal organ morphogenesis muscle organ development muscle tissue development muscle organ morphogenesis system development sarcomerogenesis cellular response to stimulus signaling intracellular signal transduction cell communication protein kinase A signaling signal transduction protein localization regulation of localization establishment of protein localization cellular localization nitrogen compound transport regulation of protein localization transport positive regulation of establishment of protein localization positive regulation of secretion by cell protein localization to extracellular region positive regulation of protein transport localization regulation of secretion by cell secretion by cell positive regulation of secretion cellular macromolecule localization regulation of transport secretion organic substance transport regulation of establishment of protein localization establishment of protein localization to extracellular region export from cell regulation of protein transport protein secretion establishment of localization positive regulation of protein secretion regulation of protein secretion regulation of secretion positive regulation of transport protein transport regulation of cellular localization macromolecule localization positive regulation of protein localization muscle hypertrophy cardiac muscle hypertrophy striated muscle hypertrophy cardiac muscle cell development cardiac cell development cardiac muscle cell differentiation cardiocyte differentiation heart process circulatory system process cardiac muscle contraction heart contraction blood circulation cardiac myofibril assembly sarcomere organization	M band intracellular anatomical structure cellular anatomical entity sarcomere intracellular organelle organelle intracellular non-membrane-bounded organelle contractile muscle fiber A band cytoplasm supramolecular fiber myofibril supramolecular polymer supramolecular complex M band intracellular anatomical structure cellular anatomical entity 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 extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle extracellular region extracellular space vesicle extracellular membrane-bounded organelle cytosol striated muscle thin filament myofilament actin cytoskeleton cytoskeleton condensed nuclear chromosome nucleus membrane-enclosed lumen nuclear chromosome chromosome intracellular membrane-bounded organelle intracellular organelle lumen organelle lumen condensed chromosome nuclear lumen
C [auth T]	TELETHONIN	cytoskeletal protein binding titin binding protein binding binding structural molecule activity structural constituent of muscle FATZ binding BMP binding cytokine binding protein-macromolecule adaptor activity molecular adaptor activity transmembrane transporter binding titin Z domain binding protein domain specific binding	cellular component organization cellular component biogenesis cellular component assembly protein-containing complex organization protein-containing complex assembly cellular process cellular component organization or biogenesis multicellular organismal process otic vesicle development sensory organ development tube formation embryo development epithelial tube formation multicellular organism development cellular component organization cellular component biogenesis cellular component assembly protein-containing complex organization protein-containing complex assembly cellular process cellular component organization or biogenesis multicellular organismal process otic vesicle development sensory organ development tube formation embryo development epithelial tube formation multicellular organism development tube morphogenesis inner ear development otic vesicle formation anatomical structure development animal organ morphogenesis epithelial tube morphogenesis embryonic organ development developmental process embryonic organ morphogenesis ear morphogenesis epithelium development tube development ear development embryonic morphogenesis animal organ development morphogenesis of an epithelium tissue development otic vesicle morphogenesis tissue morphogenesis anatomical structure morphogenesis sensory organ morphogenesis inner ear morphogenesis anatomical structure formation involved in morphogenesis cellular developmental process cellular component assembly involved in morphogenesis non-membrane-bounded organelle assembly cell development myosin filament assembly skeletal myofibril assembly muscle cell differentiation muscle structure development striated muscle cell development cellular anatomical entity morphogenesis muscle cell development cell differentiation actin filament-based process skeletal muscle myosin thick filament assembly actomyosin structure organization actin cytoskeleton organization organelle assembly myosin filament organization striated muscle myosin thick filament assembly myofibril assembly organelle organization striated muscle cell differentiation supramolecular fiber organization cytoskeleton organization heart development cardiac muscle tissue development heart morphogenesis muscle tissue morphogenesis striated muscle tissue development circulatory system development cardiac muscle tissue morphogenesis muscle organ development muscle tissue development muscle organ morphogenesis system development somitogenesis chordate embryonic development segmentation anterior/posterior pattern specification pattern specification process regionalization embryo development ending in birth or egg hatching somite development skeletal muscle thin filament assembly actin filament organization detection of external stimulus detection of stimulus response to stimulus detection of mechanical stimulus response to mechanical stimulus response to external stimulus response to abiotic stimulus detection of abiotic stimulus sarcomerogenesis multicellular organismal movement musculoskeletal movement system process nervous system process neuromuscular process skeletal muscle contraction muscle contraction muscle system process striated muscle contraction muscle hypertrophy cardiac muscle hypertrophy striated muscle hypertrophy cardiac muscle cell development cardiac cell development cardiac muscle cell differentiation cardiocyte differentiation heart process circulatory system process cardiac muscle contraction heart contraction blood circulation cardiac myofibril assembly detection of muscle stretch response to muscle stretch adult heart development sarcomere organization striated muscle adaptation muscle adaptation cardiac muscle adaptation response to stress cardiac muscle hypertrophy in response to stress muscle hypertrophy in response to stress	intracellular anatomical structure cellular anatomical entity Z disc sarcomere intracellular organelle I band organelle intracellular non-membrane-bounded organelle contractile muscle fiber cytoplasm supramolecular fiber myofibril supramolecular polymer supramolecular complex intracellular anatomical structure cellular anatomical entity Z disc sarcomere intracellular organelle I band organelle intracellular non-membrane-bounded organelle contractile muscle fiber cytoplasm supramolecular fiber myofibril supramolecular polymer supramolecular complex non-membrane-bounded organelle cytosol

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, B	IPR036179	Immunoglobulin-like domain superfamily	Homologous Superfamily
A, B	IPR008266	Tyrosine-protein kinase, active site	Active Site
A, B	IPR000719	Protein kinase domain	Domain
A, B	IPR013106	Immunoglobulin V-set domain	Domain
A, B	IPR015129	Titin, Z repeat	Repeat
A, B	IPR013783	Immunoglobulin-like fold	Homologous Superfamily
A, B	IPR007110	Immunoglobulin-like domain	Domain
A, B	IPR004168	PPAK motif	Repeat
A, B	IPR003599	Immunoglobulin subtype	Domain
A, B	IPR003598	Immunoglobulin subtype 2	Domain
A, B	IPR040849	MyBP-C, tri-helix bundle domain	Domain
A, B	IPR013098	Immunoglobulin I-set	Domain
A, B	IPR003961	Fibronectin type III	Domain
A, B	IPR011009	Protein kinase-like domain superfamily	Homologous Superfamily
A, B	IPR036116	Fibronectin type III superfamily	Homologous Superfamily
C [auth T]	IPR015667	Telethonin	Family
C [auth T]	IPR023111	Titin-like domain superfamily	Homologous Superfamily

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, B	Pharos: Q8WZ42	limb-girdle muscular dystrophy dilated cardiomyopathy myopathy myopathy, myofibrillar, 9, with early respiratory failure tibial muscular dystrophy early-onset myopathy with fatal cardiomyopathy autosomal recessive limb-girdle muscular dystrophy type 2J distal myopathy hypertrophic cardiomyopathy 9 dilated cardiomyopathy 1G atrial fibrillation familial dilated cardiomyopathy hypertrophic cardiomyopathy lung adenocarcinoma arrhythmogenic right ventricular cardiomyopathy limb-girdle muscular dystrophy dilated cardiomyopathy myopathy myopathy, myofibrillar, 9, with early respiratory failure tibial muscular dystrophy early-onset myopathy with fatal cardiomyopathy autosomal recessive limb-girdle muscular dystrophy type 2J distal myopathy hypertrophic cardiomyopathy 9 dilated cardiomyopathy 1G atrial fibrillation familial dilated cardiomyopathy hypertrophic cardiomyopathy lung adenocarcinoma arrhythmogenic right ventricular cardiomyopathy Muscle Weakness cardiomyopathy Emery-Dreifuss muscular dystrophy urinary bladder carcinoma familial isolated dilated cardiomyopathy hereditary spastic paraplegia amyotrophic lateral sclerosis lung carcinoma muscle tissue disease B-cell chronic lymphocytic leukemia muscular dystrophy colorectal adenocarcinoma ovarian mucinous adenocarcinoma squamous cell lung carcinoma gastric adenocarcinoma intellectual disability lung large cell carcinoma autosomal recessive centronuclear myopathy Becker muscular dystrophy centronuclear myopathy ovarian serous adenocarcinoma dilated cardiomyopathy 1A familial hypertrophic cardiomyopathy heart disease kidney Wilms tumor lung cancer arthrogryposis metastatic melanoma muscular disease myopathy, centronuclear, 2 Charcot-Marie-Tooth disease non-small cell lung carcinoma
C [auth T]	Pharos: O15273	hypertrophic cardiomyopathy 25 limb-girdle muscular dystrophy dilated cardiomyopathy autosomal recessive limb-girdle muscular dystrophy type 2G cardiomyopathy myopathy hypertrophic cardiomyopathy large cell medulloblastoma malignant glioma medulloblastoma muscular dystrophy pilocytic astrocytoma arthrogryposis rheumatoid arthritis bipolar disorder hypertrophic cardiomyopathy 25 limb-girdle muscular dystrophy dilated cardiomyopathy autosomal recessive limb-girdle muscular dystrophy type 2G cardiomyopathy myopathy hypertrophic cardiomyopathy large cell medulloblastoma malignant glioma medulloblastoma muscular dystrophy pilocytic astrocytoma arthrogryposis rheumatoid arthritis bipolar disorder Cardiomyopathy, Hypertrophic, Familial congenital muscular dystrophy familial isolated dilated cardiomyopathy malignant mesothelioma muscle tissue disease primary biliary cholangitis atypical teratoid rhabdoid tumor systemic lupus erythematosus Charcot-Marie-Tooth disease Crohn disease familial dilated cardiomyopathy glioblastoma inflammatory bowel disease lung adenocarcinoma marginal zone lymphoma muscular disease primitive neuroectodermal tumor ulcerative colitis dermatomyositis familial hypertrophic cardiomyopathy

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.