Gigantic business: titin properties and function through thick and thin

WA Linke, N Hamdani - Circulation research, 2014 - Am Heart Assoc
The giant protein titin forms a unique filament network in cardiomyocytes, which engages in
both mechanical and signaling functions of the heart. TTN, which encodes titin, is also a …

The sarcomeric cytoskeleton: from molecules to motion

M Gautel, K Djinović-Carugo - Journal of Experimental …, 2016 - journals.biologists.com
Highly ordered organisation of striated muscle is the prerequisite for the fast and
unidirectional development of force and motion during heart and skeletal muscle …

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

B Gerull, M Gramlich, J Atherton, M McNabb… - Nature …, 2002 - nature.com
Congestive heart failure (CHF) can result from various disease states with inadequate
cardiac output. CHF due to dilated cardiomyopathy (DCM) is a familial disease in 20–30% of …

A Rising Titan: TTN Review and Mutation Update

C Chauveau, J Rowell, A Ferreiro - Human mutation, 2014 - Wiley Online Library
The 364 exon TTN gene encodes titin (TTN), the largest known protein, which plays key
structural, developmental, mechanical, and regulatory roles in cardiac and skeletal muscles …

Striated muscle cytoarchitecture: an intricate web of form and function

KA Clark, AS McElhinny, MC Beckerle… - Annual review of cell …, 2002 - annualreviews.org
▪ Abstract Striated muscle is an intricate, efficient, and precise machine that contains
complex interconnected cytoskeletal networks critical for its contractile activity. The …

Tibial muscular dystrophy is a titinopathy caused by mutations in TTN, the gene encoding the giant skeletal-muscle protein titin

P Hackman, A Vihola, H Haravuori, S Marchand… - The American Journal of …, 2002 - cell.com
Tibial muscular dystrophy (TMD) is an autosomal dominant late-onset distal myopathy linked
to chromosome 2q31. The linked region includes the giant TTN gene, which encodes the …

The structure and regulation of human muscle α-actinin

E de Almeida Ribeiro, N Pinotsis, A Ghisleni… - Cell, 2014 - cell.com
The spectrin superfamily of proteins plays key roles in assembling the actin cytoskeleton in
various cell types, crosslinks actin filaments, and acts as scaffolds for the assembly of large …

Costameres: the Achilles' heel of Herculean muscle

JM Ervasti - Journal of Biological Chemistry, 2003 - ASBMB
Like the ancient Greek hero Hercules, striated muscle is famous for performing impressive
feats of strength. The procurement of food, breathing, and defense or escape from harm all …

Developmental control of titin isoform expression and passive stiffness in fetal and neonatal myocardium

S Lahmers, Y Wu, DR Call, S Labeit… - Circulation …, 2004 - Am Heart Assoc
Developmental changes in contractile behavior are known to occur during fetal and
postnatal heart development. In this study, we examined whether adaptations take place in …

The vertebrate muscle Z-disc: sarcomere anchor for structure and signalling

PK Luther - Journal of muscle research and cell motility, 2009 - Springer
The Z-disc, appearing as a fine dense line forming sarcomere boundaries in striated
muscles, when studied in detail reveals crosslinked filament arrays that transmit tension and …