iPSCs for cardiomyopathy modeling
By: Ipscs Progress
Tags: Health, Stem Cell Research, STEM, Heart, Biotechnology

iPSCs for cardiomyopathy modeling

Cardiomyopathy is a disease of the heart muscle. In most cases, cardiomyopathy causes the heart muscle enlargement, thickness or rigidity. In rare instances, scar tissue replaces diseased heart muscle tissue. Usually, no signs or symptoms in the early stages. As the condition advances, the heart hard to pump blood throughout the body. As a result, the weak heart unable to maintain daily work lead to heart failure or irregular heartbeats. Sometimes also accompanied with other heart valve problem.

Causes and Symptoms

Often the underlying cause of the cardiomyopathy is unknown. Diabetes, long-term alcoholism, long-term high blood pressure, heart attack or other diseases might raise the risk of cardiomyopathy. In addition to a genetic cause, some might correspond to inherited reason. No symptoms are characterized in the early stages of cardiomyopathy. But when the condition worsens, signs and symptoms usually appear, including breathlessness after physical exertion, cough, fatigue, swelling of the legs, ankles, chest discomfort or abdomen. If untreated, signs and symptoms tend to get worse and even lead to sudden cardiac death.

Types of cardiomyopathy

The main types of cardiomyopathy include dilated, hypertrophic, restrictive cardiomyopathy, arrhythmogenic RV and other unclassified types.

Dilated cardiomyopathy.

In dilated cardiomyopathy, the ventricles enlarge and weaken that can’t effectively pump blood out of the heart. The most common cause of orthotopic heart transplantation in infants and young adults. The most common cause is coronary artery disease or heart attack.

Hypertrophic cardiomyopathy.

In this type, the heart muscle enlarges and thickens and results in abnormal heart work. Most prevalent inherited cardiovascular disease and link with some genetic mutation. One of the most common causes of sudden cardiac death in young adults.

Restrictive Cardiomyopathy.

In this type, the heart muscle stiffens and can not expand. This least common type of cardiomyopathy can occur at any age, but it most prevalent in older people. The cause of this type can occur idiopathically or it can be caused by a disease elsewhere in the body that affects the heart.

Arrhythmogenic right ventricular dysplasia.

In this rare type of cardiomyopathy, the muscle in the lower right heart chamber (right ventricle) is replaced by scar tissue, which can lead to heart rhythm problems. It’s often caused by genetic mutations.

Unclassified cardiomyopathy.

Other types of cardiomyopathy fall into this category.

Treatment

The goals of cardiomyopathy treatment are to prevent symptoms worsening, improve symptoms, and reduce the risk of complications. Treatment varies by the type of cardiomyopathy. General treatment might be a healthy lifestyle change, regular medication, and surgical treatment.

Lifestyle changes

These include eating a low-fat and low-sodium diet, getting moderate exercise, and limiting smoking and alcohol.

Medications

The doctor may prescribe medications to improve heart ability if lifestyle changes not work. The type of medication should be specific to cardiomyopathy type

Surgery

For sever cardiomyopathy, specific procedures or surgery might be recommended.

iPSC-derived cardiomyocytes

iPSC-derived cardiomyocytes as an in vitro research model allow scientists to build their own disease models specific to their research. iPSC-derived cardiomyocytes are ideal for disease modeling, Drug toxicity testing as well as cardiac regeneration therapeutics discovery. iPSC is highly organized and could culture spontaneously beat in vitro. iPSC-derived cardiomyocytes can also express the cardiac-specific markers (eg. cardiac troponin).

Application of iPSC-derived cardiomyocyte

Characteristics of iPSC-Cardiomyocytes

By modulation the signaling pathways involved in cardiomyocytes differentiation, iPSC could divide to cardiomyocytes. iPSC-derived cardiomyocytes cultures generate spontaneously beating cells. In a mature culture, the cells begin to segregate and could become subtype specific (ventricular, atrial or nodal). The cardiomyocytes derived from iPSC also maintain the functional phenotype in culture. IPSC-cardiomyocytes have a similar characteristic as adult cardiomyocytes that express major cardiac ion channel proteins and sarcomeric proteins, exhibit action potentials and react to calcium.

Disease Modeling using iPSC-Cardiomyocytes

The advent of iPSC technology for human cells in 2007 opened up new possibilities in cardiovascular research. By using this technology, an unlimited number of cells could be obtained and have less ethical controversy compared with human embryonic cells. The use of iPSC-derived cardiomyocytes for human disease modeling takes advantage of a system that provides a continuous source and allows the long-term assessment. iPSC-derived cardiomyocytes also complement the drawbacks for using heterogeneous sources such as primary cells. iPSC-derived cardiomyocytes could help study either healthy donors or in disease and genetically relevant background in vitro. iPS cells provide a valuable tool for the study of heart disease as cardiomyopathy