Connectin

A giant elastic protein of molecular mass ranging from 2,993 kDa (cardiac), 3,300 kDa (psoas), to 3,700 kDa (soleus) having a kinase domain. The amino- terminal is involved in a Z line binding, and the carboxy-terminal region is bound to the myosin filament with an overlap between the counter-connectin filaments at the M line.

Also Known As:

M-Band Proteins; M-Line 185 kDa Protein; M-Protein (muscle); Muscle M-Line Protein; Myomesin; Skelemins; Titin; Titin 1; Titin 2; Titin Kinase; alpha-Connectin; beta-Connectin; Kinase, Titin; M Band Proteins; M Line 185 kDa Protein; M-Line Protein, Muscle; Muscle M Line Protein; Protein, Muscle M-Line; Proteins, M-Band; alpha Connectin; beta Connectin

Networked: 677 relevant articles (12 outcomes, 54 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Linke, Wolfgang A: 38 articles (11/2022 - 09/2002)
2.	Granzier, Henk: 36 articles (01/2021 - 02/2002)
3.	Labeit, Siegfried: 30 articles (01/2022 - 02/2002)
4.	Udd, Bjarne: 25 articles (10/2020 - 09/2002)
5.	Hamdani, Nazha: 22 articles (01/2022 - 01/2008)
6.	Granzier, Henk L: 16 articles (01/2022 - 07/2003)
7.	Hackman, Peter: 14 articles (01/2020 - 09/2002)
8.	Gotthardt, Michael: 13 articles (01/2022 - 02/2003)
9.	Mestroni, Luisa: 13 articles (01/2022 - 08/2011)
10.	Cook, Stuart A: 13 articles (01/2019 - 02/2012)

Related Diseases

1.	Hypertrophy 12/01/2008 - "Mechanistic studies demonstrated that FHL1 plays an important role in the mechanism of pathological hypertrophy by sensing biomechanical stress responses via the N2B stretch sensor domain of titin and initiating changes in the titin- and MAPK-mediated responses important for sarcomere extensibility and intracellular signaling. " 06/01/2020 - "The mdm phenotype also points to the existence of as yet unidentified signaling pathways for muscle hypertrophy and thermoregulation, likely involving titin's PEVK region as well as the N2A signalosome." 01/01/2020 - "Deleting Titin's C-Terminal PEVK Exons Increases Passive Stiffness, Alters Splicing, and Induces Cross-Sectional and Longitudinal Hypertrophy in Skeletal Muscle." 12/19/2018 - "Thus, skeletal muscle titin is the dominant determinant of physiological passive stiffness and drives longitudinal hypertrophy. " 10/01/2018 - "Thus, muscle hypertrophy scales with titin stiffness. "
2.	Fibrosis (Cirrhosis) 01/01/2021 - "Growing literature supports the presence of abnormalities in titin and its phosphorylation, and increased interstitial fibrosis contributing to increased chamber stiffness. " 01/01/2020 - "Cardiomyocyte contractile properties and fibrosis were comparable between stage II and IV, while capillary density was significantly lower in stage IV compared to stage II. Women showed more adverse cellular remodeling compared to men at stage II, evident from more compliant titin, more fibrosis and lower capillary density. " 12/01/2019 - "We further demonstrate that increased cardiac titin phosphorylation and reactive interstitial fibrosis associated with neutrophil extracellular traps lead to left ventricular stiffness and suggest that both pathways may be potential therapeutic targets in Asian HFpEF patients." 01/01/2018 - "At a cellular level, HCM women showed increased compliant titin and a larger degree of interstitial fibrosis." 01/01/2018 - "In female patients with HCM, titin was more compliant, and more fibrosis was present compared with men. "
3.	Dilated Cardiomyopathy (Cardiomyopathy, Congestive) 10/31/2017 - "Improved understanding of dilated cardiomyopathy (DCM) due to titin truncation (TTNtv) may help guide patient stratification. " 10/01/2004 - "In this study, we analyzed titin expression in left ventricles of 19 control human donors and 9 severely diseased (nonischemic) dilated cardiomyopathy (DCM) transplant-patients, using gel-electrophoresis, immunoblotting, and quantitative RT-PCR. " 10/01/2023 - "Titin-truncating variants (TTNtv) are the single largest genetic cause of dilated cardiomyopathy (DCM). " 05/23/2023 - "Truncating variants in titin (TTNtv) are the most prevalent genetic etiology of dilated cardiomyopathy (DCM). " 02/15/2023 - "The mutations of TTN gene that encodes titin are the most common mutation type among the genetic causes of dilated cardiomyopathy (DCM). "
4.	Heart Diseases (Heart Disease) 01/01/2017 - "This review highlights advances in understanding the relationship between molecular (titin-based) and in vivo (left ventricular) passive stiffness, how passive stiffness modifies exercise tolerance, and how exercise training may be therapeutic for cardiac diseases with increased passive stiffness. " 11/10/2022 - "Due to the enormous size, complexity, and malleability of the titin molecule, titin properties are also vulnerable to dysregulation, as observed in various cardiac disorders. " 03/07/2022 - "Furthermore, EH-myomesin apart from its role during embryonic stages, is present in adults with specific cardiac diseases. " 01/01/2022 - "These findings define a role for a deacetylase in the control of myofibril function and myocardial passive stiffness, suggest that reversible acetylation alters titin compliance, and reveal the potential of targeting HDAC6 to manipulate the elastic properties of the heart to treat cardiac diseases." 01/01/2022 - "Our results demonstrate the role of conserved cysteines in the modulation of titin mechanical properties in vivo and point to potential redox-based pathomechanisms in heart disease."
5.	Thymoma (Thymic Carcinoma) 04/01/2020 - "Anti-AChR was detected in al all patients associated with anti-Titin and -RyR1 in those patients with thymoma. " 09/01/2010 - "Titin antibody combined with computed tomography was better for the diagnosis of thymoma. " 07/01/2010 - "Serum antititin antibody levels were closely related to titin MIR levels in thymoma cells; however, titin MIR levels did not appear to be related to MG severity. " 07/21/2009 - "The rates of relapse and conversion in OMG with Titin Ab positive or thymomas were higher than OMG with Titin Ab negative or non-thymomas (Log-Rank, P < 0.05). " 07/01/2002 - "In MG-associated thymoma, the mechanisms involved in the initial autosensitization against titin and RyR are probably similar to those implicated in the autosensitization against AChR. "

Related Drugs and Biologics

1.	Proteins (Proteins, Gene)
2.	Antibodies
3.	Protein Isoforms (Isoforms)
4.	Matrix Metalloproteinases (MMPs)
5.	Desmin
6.	Troponin T (Troponin T1)
7.	calpastatin
8.	Interleukin-12 (IL 12)
9.	Caspase 3 (Caspase-3)
10.	Proteasome Endopeptidase Complex (Proteasome)

Related Therapies and Procedures

1.	Protein-Restricted Diet (Diet, Protein Restricted)
2.	Denervation
3.	Therapeutics
4.	Thymectomy
5.	Ligation