The Giant Protein Titin

Identification of Ubiquitin Ligases Required for Skeletal Muscle Atrophy

2001 • 3,292 citations

Folding-Unfolding Transitions in Single Titin Molecules Characterized with Laser Tweezers

1997 • 1,198 citations

Titins: Giant Proteins in Charge of Muscle Ultrastructure and Elasticity

1995 • 1,195 citations

The Genetic Basis for Cardiomyopathy

2001 • 1,049 citations

Mutations in the proteolytic enzyme calpain 3 cause limb-girdle muscular dystrophy type 2A

1995 • 976 citations

The Cardiac Mechanical Stretch Sensor Machinery Involves a Z Disc Complex that Is Defective in a Subset of Human Dilated Cardiomyopathy

2002 • 791 citations

The Complete Gene Sequence of Titin, Expression of an Unusual ≈700-kDa Titin Isoform, and Its Interaction With Obscurin Identify a Novel Z-Line to I-Band Linking System

2001 • 640 citations

Passive tension in cardiac muscle: contribution of collagen, titin, microtubules, and intermediate filaments

1995 • 610 citations

The organization of titin filaments in the half-sarcomere revealed by monoclonal antibodies in immunoelectron microscopy: a map of ten nonrepetitive epitopes starting at the Z line extends close to the M line.

1988 • 608 citations

Mutations of TTN, encoding the giant muscle filament titin, cause familial dilated cardiomyopathy

2002 • 589 citations

Titin: major myofibrillar components of striated muscle.

1979 • 523 citations

Reverse engineering of the giant muscle protein titin

2002 • 505 citations

X-ray diffraction measurements of the extensibility of actin and myosin filaments in contracting muscle

1994 • 472 citations

X-ray diffraction evidence for the extensibility of actin and myosin filaments during muscle contraction

1994 • 464 citations

Stress Pathways and Heart Failure

1999 • 463 citations

Tibial Muscular Dystrophy Is a Titinopathy Caused by Mutations in TTN, the Gene Encoding the Giant Skeletal-Muscle Protein Titin

2002 • 451 citations

Muscle LIM protein, a novel essential regulator of myogenesis, promotes myogenic differentiation

1994 • 451 citations

Differential Expression of Cardiac Titin Isoforms and Modulation of Cellular Stiffness

2000 • 403 citations

Identification of muscle specific ring finger proteins as potential regulators of the titin kinase domain

2001 • 383 citations

Calcium-dependent molecular spring elements in the giant protein titin

2003 • 370 citations

Sequence of an unusually large protein implicated in regulation of myosin activity in C. elegans

1989 • 368 citations

Titin Isoform Switch in Ischemic Human Heart Disease

2002 • 368 citations

Series of Exon-Skipping Events in the Elastic Spring Region of Titin as the Structural Basis for Myofibrillar Elastic Diversity

2000 • 367 citations

Structural basis for activation of the titin kinase domain during myofibrillogenesis

1998 • 355 citations

Towards a molecular understanding of titin.

1992 • 338 citations

The Muscle Ankyrin Repeat Proteins: CARP, ankrd2/Arpp and DARP as a Family of Titin Filament-based Stress Response Molecules

2003 • 334 citations

Muscle-specific Calpain, p94, Responsible for Limb Girdle Muscular Dystrophy Type 2A, Associates with Connectin through IS2, a p94-specific Sequence

1995 • 325 citations

Muscle assembly: a titanic achievement?

1999 • 323 citations

Regulation of skeletal muscle stiffness and elasticity by titin isoforms: a test of the segmental extension model of resting tension.

1991 • 314 citations

The NH2 Terminus of Titin Spans the Z-Disc: Its Interaction with a Novel 19-kD Ligand (T-cap) Is Required for Sarcomeric Integrity

1998 • 305 citations

Protein Kinase A Phosphorylates Titin’s Cardiac-Specific N2B Domain and Reduces Passive Tension in Rat Cardiac Myocytes

2002 • 301 citations

Developmental Control of Titin Isoform Expression and Passive Stiffness in Fetal and Neonatal Myocardium

2004 • 296 citations

Vertical agarose gel electrophoresis and electroblotting of high‐molecular‐weight proteins

2003 • 295 citations

Reversible unfolding of fibronectin type III and immunoglobulin domains provides the structural basis for stretch and elasticity of titin and fibronectin.

1994 • 294 citations

Myopalladin, a Novel 145-Kilodalton Sarcomeric Protein with Multiple Roles in Z-Disc and I-Band Protein Assemblies

2001 • 288 citations

Muscle LIM Protein Promotes Myogenesis by Enhancing the Activity of MyoD

1997 • 286 citations

Towards a Molecular Understanding of the Elasticity of Titin

1996 • 284 citations

Obscurin, a giant sarcomeric Rho guanine nucleotide exchange factor protein involved in sarcomere assembly

2001 • 280 citations

CARP, a cardiac ankyrin repeat protein, is downstream in the Nkx2-5 homeobox gene pathway

1997 • 273 citations

Muscle-specific RING finger-1 interacts with titin to regulate sarcomeric M-line and thick filament structure and may have nuclear functions via its interaction with glucocorticoid modulatory element binding protein-1

2002 • 261 citations

Titin Develops Restoring Force in Rat Cardiac Myocytes

1996 • 250 citations

Subcellular targeting of metabolic enzymes to titin in heart muscle may be mediated by DRAL/FHL-2

2002 • 249 citations

Titin Mutations as the Molecular Basis for Dilated Cardiomyopathy

2002 • 249 citations

Titin-Based Modulation of Calcium Sensitivity of Active Tension in Mouse Skinned Cardiac Myocytes

2001 • 248 citations

Connectin/titin, giant elastic protein of muscle

1997 • 240 citations

Titin Extensibility In Situ: Entropic Elasticity of Permanently Folded and Permanently Unfolded Molecular Segments

1998 • 237 citations

I-Band Titin in Cardiac Muscle Is a Three-Element Molecular Spring and Is Critical for Maintaining Thin Filament Structure

1999 • 237 citations

Connectin, an Elastic Protein of Muscle<subtitle>Characterization and Function<xref ref-type="fn" rid="fn1"><sup>1</sup></xref></subtitle>

1977 • 231 citations

Titin–Actin Interaction in Mouse Myocardium: Passive Tension Modulation and Its Regulation by Calcium/S100A1

2001 • 221 citations

The structure of the sarcomeric M band: localization of defined domains of myomesin, M-protein, and the 250-kD carboxy-terminal region of titin by immunoelectron microscopy.

1996 • 212 citations

Changes in Titin and Collagen Underlie Diastolic Stiffness Diversity of Cardiac Muscle

2000 • 211 citations

Passive and active tension in single cardiac myofibrils

1994 • 204 citations

Titin and nebulin: protein rulers in muscle?

1994 • 202 citations

Unfolding of Titin Domains Explains the Viscoelastic Behavior of Skeletal Myofibrils

2001 • 201 citations

Binding of an ankyrin-1 isoform to obscurin suggests a molecular link between the sarcoplasmic reticulum and myofibrils in striated muscles

2003 • 187 citations

Alterations in the Determinants of Diastolic Suction During Pacing Tachycardia

2000 • 187 citations

Calsarcin-3, a Novel Skeletal Muscle-specific Member of the Calsarcin Family, Interacts with Multiple Z-disc Proteins

2002 • 187 citations

Length Dependence of Tension Generation in Rat Skinned Cardiac Muscle

2001 • 180 citations

Changes in Titin Isoform Expression in Pacing-Induced Cardiac Failure Give Rise to Increased Passive Muscle Stiffness

2002 • 176 citations

Regulation of Microtubule Dynamics and Myogenic Differentiation by Murf, a Striated Muscle Ring-Finger Protein

2000 • 171 citations

Mechanical properties of isolated cardiac myocytes

1991 • 170 citations

Mechanically Driven Contour-Length Adjustment in Rat Cardiac Titin’s Unique N2B Sequence

1999 • 165 citations

Passive force generation and titin isoforms in mammalian skeletal muscle

1992 • 160 citations

Titin/connectin and nebulin: Giant protein rulers of muscle structure and function

1996 • 158 citations

Interaction Between PEVK-Titin and Actin Filaments

2001 • 157 citations

Two immunoglobulin‐like domains of the Z‐disc portion of titin interact in a conformation‐dependent way with telethonin

1998 • 155 citations

Visualization of the polarity of isolated titin molecules: a single globular head on a long thin rod as the M band anchoring domain?

1989 • 153 citations

Active state in cardiac muscle.

1968 • 153 citations

Extensible and Less-Extensible Domains of Connectin Filaments in Stretched Vertebrate Skeletal Muscle Sarcomeres as Detected by Immunofluorescence and Immunoelectron Microscopy Using Monoclonal Antibodies1

1988 • 151 citations

Association of the Chaperone αB-crystallin with Titin in Heart Muscle

2004 • 151 citations

Conditional Expression of Mutant M-line Titins Results in Cardiomyopathy with Altered Sarcomere Structure

2003 • 138 citations

Transient association of titin and myosin with microtubules in nascent myofibrils directed by the MURF2 RING-finger protein

2002 • 137 citations

Specific interaction of the potassium channel β-subunit minK with the sarcomeric protein T-cap suggests a T-tubule-myofibril linking system

2001 • 135 citations

Molecular Mechanics of Cardiac Titin's PEVK and N2B Spring Elements

2002 • 135 citations

Passive tension and stiffness of vertebrate skeletal and insect flight muscles: the contribution of weak cross-bridges and elastic filaments

1993 • 135 citations

The Muscular Dystrophy with Myositis (mdm) Mouse Mutation Disrupts a Skeletal Muscle-Specific Domain of Titin

2002 • 133 citations

The Mechanically Active Domain of Titin in Cardiac Muscle

1995 • 132 citations

Ca2+ /S100 regulation of giant protein kinases

1996 • 132 citations

An internal viscous element limits unloaded velocity of sarcomere shortening in rat myocardium.

1992 • 131 citations

Cardiac Ankyrin Repeat Protein Is a Novel Marker of Cardiac Hypertrophy

2000 • 131 citations

Identification of Ankrd2, a Novel Skeletal Muscle Gene Coding for a Stretch-Responsive Ankyrin-Repeat Protein

2000 • 129 citations

Control of segmental expression of the cardiac-restricted ankyrin repeat protein gene by distinct regulatory pathways in murine cardiogenesis

1999 • 125 citations

Titin and the sarcomere symmetry paradox11Edited by J. Karn

2001 • 123 citations

Titin Isoform Variance and Length Dependence of Activation in Skinned Bovine Cardiac Muscle

2003 • 122 citations

Titin isoform expression in normal and hypertensive myocardium

2003 • 120 citations

Connectin, an elastic protein of striated muscle

1994 • 116 citations

Muscle-Specific Calpain, p94, Interacts with the Extreme C-Terminal Region of Connectin, a Unique Region Flanked by Two Immunoglobulin C2 Motifs

1997 • 115 citations

Titin: a molecular control freak

1999 • 115 citations

Titin elasticity and mechanism of passive force development in rat cardiac myocytes probed by thin-filament extraction

1997 • 113 citations

A Non-Cross-Bridge Stiffness in Activated Frog Muscle Fibers

2002 • 112 citations

A molecular map of titin/connectin elasticity reveals two different mechanisms acting in series

1996 • 112 citations

Titin Determines the Frank-Starling Relation in Early Diastole

2003 • 112 citations

Phosphorylation of KSP motifs in the C-terminal region of titin in differentiating myoblasts.

1993 • 111 citations

Possible Regulation of the Conventional Calpain System by Skeletal Muscle-specific Calpain, p94/Calpain 3

2004 • 110 citations

Titin‐cap associates with, and regulates secretion of, Myostatin

2002 • 108 citations

Genomic Organization of M Line Titin and its Tissue-specific Expression in Two Distinct Isoforms

1996 • 101 citations

A Spring Tale: New Facts on Titin Elasticity

1998 • 97 citations

Mechanical Fatigue in Repetitively Stretched Single Molecules of Titin

2001 • 95 citations

Structural and functional studies of titin’s fn3 modules reveal conserved surface patterns and binding to myosin S1 - a possible role in the frank-starling mechanism of the heart

2001 • 95 citations

Molecular Dissection of N2B Cardiac Titin’s Extensibility

1999 • 91 citations

Induction and Myofibrillar Targeting of CARP, and Suppression of the Nkx2.5 Pathway in the MDM Mouse with Impaired Titin-based Signaling

2003 • 89 citations

Identification, tissue expression and chromosomal localization of human Obscurin-MLCK, a member of the titin and Dbl families of myosin light chain kinases

2002 • 89 citations

Cardiac titin isoforms are coexpressed in the half-sarcomere and extend independently

2001 • 88 citations

Length Modulation of Active Force in Rat Cardiac Myocytes: is Titin the Sensor?

1999 • 87 citations

Identification of new repeating motifs in titin

2001 • 84 citations

Arpp, a New Homolog of Carp, Is Preferentially Expressed in Type 1 Skeletal Muscle Fibers and Is Markedly Induced by Denervation

2002 • 77 citations

Cytoskeletal Matrix in Striated Muscle: The Role of Titin, Nebulin and Intermediate Filaments

1984 • 73 citations

The Elastic I-band Region of Titin is Assembled in a "Modular" Fashion by Weakly Interacting Ig-like Domains

1996 • 72 citations

The Hydrophilic Domain of Small Ankyrin-1 Interacts with the Two N-terminal Immunoglobulin Domains of Titin

2003 • 67 citations

Molecular Identification and Characterization of a Novel Nuclear Protein Whose Expression Is Up-regulated in Insulin-resistant Animals

2003 • 67 citations

The anatomy of a molecular giant: How the sarcomere cytoskeleton is assembled from immunoglobulin superfamily molecules

1995 • 64 citations

Characterization of a 5.4 kb cDNA fragment from the Z-line region of rabbit cardiac titin reveals phosphorylation sites for proline-directed kinases

1995 • 63 citations

Downregulation and Nuclear Relocation of MLP During the Progression of Right Ventricular Hypertrophy Induced by Chronic Pressure Overload

2000 • 62 citations

Autophosphorylation of molluscan twitchin and interaction of its kinase domain with calcium/calmodulin.

1994 • 61 citations

The physiological role of titin in striated muscle

1999 • 60 citations

Muscle Z-band Ultrastructure: Titin Z-repeats and Z-band Periodicities Do Not Match

2002 • 59 citations

Cotranslational assembly of some cytoskeletal proteins: implications and prospects

1993 • 58 citations

Thick filament movement and isometric tension in activated skeletal muscle

1988 • 52 citations

Mechanical Properties of Titin Isoforms

2000 • 48 citations

Both synchronous and asynchronous muscle isoforms of projectin (the Drosophila bent locus product) contain functional kinase domains.

1995 • 48 citations

Titin Domain Patterns Correlate with the Axial Disposition of Myosin at the End of the Thick Filament

1996 • 46 citations

A Calmodulin‐binding Sequence in the C‐terminus of Human Cardiac Titin Kinase

1995 • 45 citations

Molecular Basis of Passive Stress Relaxation in Human Soleus Fibers: Assessment of the Role of Immunoglobulin-Like Domain Unfolding

2003 • 44 citations

Cardiac ankyrin repeat protein is preferentially induced in atrophic myofibers of congenital myopathy and spinal muscular atrophy

2003 • 44 citations

A 11.5-kb 5′-Terminal cDNA Sequence of Chicken Breast Muscle Connectin/Titin Reveals Its Z Line Binding Region

1996 • 39 citations

Cycling Cross-Bridges Increase Myocardial Stiffness at Submaximal Levels of Ca2+ Activation

2003 • 36 citations

Species variations in cDNA sequence and exon splicing patterns in the extensible I-band region of cardiac titin: relation to passive tension.

2002 • 33 citations

In vivo phosphorylation of titin and nebulin in frog skeletal muscle

1987 • 31 citations

Z-line/I-band and A-band lattices of intact frog sartorius muscle at altered interfilament spacing

1992 • 21 citations

Degradation of connectin (titin) in Fukuyama type congenital muscular dystrophy: immunochemical study with monoclonal antibodies

1990 • 13 citations

Diastolic Viscoelastic Properties of Rat Cardiac Muscle; Involvement of Ca2+

1997 • 9 citations