Related Experiment Video

Updated: May 9, 2026

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
03:45

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Published on: August 8, 2022

Deficiency of cartilage oligomeric matrix protein causes dilated cardiomyopathy

Yaqian Huang1, Jiahong Xia, Jingang Zheng

  • 1Department of Physiology and Pathophysiology, Basic Medical College of Peking University, Peking University, Beijing, 100191, People's Republic of China.

Basic Research in Cardiology
|August 7, 2013
PubMed

Insights

Cartilage oligomeric matrix protein (COMP) deficiency causes dilated cardiomyopathy (DCM) in mice. COMP maintains cardiac homeostasis by interacting with integrin β1, suggesting a therapeutic target for DCM.

Area of Science:

  • Cardiovascular Biology
  • Extracellular Matrix Research
  • Integrin Signaling

Background:

  • Cardiac extracellular matrix alterations are implicated in dilated cardiomyopathy (DCM) and heart failure.
  • The matricellular protein cartilage oligomeric matrix protein (COMP) is present in the heart, but its function is unclear.

Purpose of the Study:

  • To investigate the role of COMP in cardiac homeostasis and its involvement in DCM.

Main Methods:

  • Utilized COMP knockout (COMP-/-) mice to assess cardiac function and structure.
  • Analyzed cardiac tissue from COMP-/- mice and human DCM patient samples.
  • Investigated the interaction between COMP and integrin β1 signaling pathways.

Main Results:

  • COMP-/- mice developed spontaneous DCM with impaired cardiac function and cardiac ultrastructure defects.
  • COMP deficiency led to cardiomyocyte apoptosis, myofilament loss, connexin-43 deficiency, and matrix metalloproteinase activation.
  • Reduced COMP expression was found in DCM patients; COMP directly binds integrin β1, preventing its degradation and maintaining cardiac homeostasis.

Conclusions:

  • COMP is essential for maintaining cardiac homeostasis.
  • The COMP-integrin β1 axis is a critical pathway in preventing DCM and represents a potential therapeutic target.

More Related Videos

A Doxorubicin-Induced Murine Model of Dilated Cardiomyopathy In Vivo05:14

A Doxorubicin-Induced Murine Model of Dilated Cardiomyopathy In Vivo

Published on: May 16, 2020

Establishment and Evaluation of a Sheep Model of Full-thickness Osteochondral Defect05:23

Establishment and Evaluation of a Sheep Model of Full-thickness Osteochondral Defect

Published on: April 14, 2026

Related Concept Videos

Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
Cardiomyopathy V: Interprofessional Care01:29

Cardiomyopathy V: Interprofessional Care

Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
Mitral Valve Prolapse I: Introduction01:27

Mitral Valve Prolapse I: Introduction

IntroductionThe mitral valve, one of the heart's four valves, regulates blood flow. These valves have flaps that open and close to direct blood properly through the heart and body. During each heartbeat, the flaps open for blood to pass through and seal shut to prevent backflow. Specifically, the mitral valve opens to allow blood flow from the heart's upper left chamber to the lower left chamber. It then closes securely as the lower left chamber contracts to pump blood to the body, preventing...
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...