NYMC Faculty Publications

Titin: The Missing Link in Cardiac Physiology

Author Type(s)

Faculty

DOI

10.1097/CRD.0000000000000656

Journal Title

Cardiology in Review

First Page

47

Last Page

52

Document Type

Article

Publication Date

1-1-2026

Department

Medicine

Keywords

dilated cardiomyopathy, familial restrictive cardiomyopathy, hypertrophic cardiomyopathy, left ventricular noncompaction cardiomyopathy, titin

Disciplines

Medicine and Health Sciences

Abstract

Titin, an extraordinary protein known for its colossal size and multifaceted roles, is a cornerstone in the structural and functional dynamics of striated muscle tissues, including the heart and skeletal muscles. Its sheer enormity, with a molecular weight exceeding 3000 kDa, is paralleled only by the immense influence it exerts on muscle physiology. This review will delve into the remarkable structural organization of Titin and the genetics of this molecule, including the common mutations resulting in various cardiomyopathies. We will delve deeper into its role in dilated cardiomyopathy, familial restrictive cardiomyopathy, hypertrophic cardiomyopathy, and left ventricular noncompaction cardiomyopathy. This review culminates by discussing the prospects of therapeutic strategies targeting Titin. While these interventions remain primarily theoretical, the possibilities are intriguing. Patients with Titin truncation mutations present unique challenges, but innovative approaches like gene therapy or preemptive treatments with drugs such as angiotensin-converting enzyme inhibitors or beta-blockers offer hope. This multi-pronged approach highlights the significance of understanding Titin's multifaceted role and its potential as a target for future therapeutic interventions.

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