Restrictive Cardiomyopathy

Restrictive cardiomyopathy has been defined as a failure of the left and/or right ventricle to dilate properly with associated diastolic dysfunction. Three major causes of restrictive cardiomyopathy have specific therapy options: cardiac amyloidosis, cardiac sarcoidosis, and cardiac hemochromatosis. Protheragen is dedicated to understanding rare cardiovascular disorders through extensive research, and novel methods, and serves as a comprehensive drug development solution to researchers globally.

Introduction to Restrictive Cardiomyopathy

The least common type of cardiomyopathy is restrictive cardiomyopathy, which is characterized by increased stiffness of the myocardium, leading to impaired ventricle filling. Most individuals exhibit normal or almost normal biventricular systolic function and chamber size until symptoms worsen in the later stages of the disease. Both ventricles may be impacted, resulting in some degree of left or right heart failure. The occurrence of arrhythmias and conduction disturbances is rather common.

Fig.1 Classification of restrictive cardiomyopathy. (Rapezzi, C., et al., 2022)

Pathogenesis of Restrictive Cardiomyopathy

Restrictive cardiomyopathy may stem from a genetic predisposition or a combination of disease processes, which can be classified broadly as infiltrative, storage disease, noninfiltrative, and endomyocardial. Several gene mutations have been mapped out as one cause of restrictive cardiomyopathy. These include sarcomere subunit mutations like those in troponin T (TNNT2), troponin I (TNNI3), α-actin (ACTC), and β-myosin heavy chain (MYH7). A majority of these mutations are passed down through families in an autosomal dominant manner.

Fig.2 Myocardial tissue of nine different subtypes of restrictive cardiomyopathy. (Rapezzi, C., et al., 2022)

Therapeutics Development for Restrictive Cardiomyopathy

Disclaimer: Protheragen focuses on providing preclinical research services. This table is for information exchange purposes only. This table is not a therapy plan recommendation. For guidance on therapy options, please visit a regular hospital.

Our Services

Understanding the difficulties posed by restrictive cardiomyopathy as a rare cardiovascular disease, we develop research methodologies to fit those specific gaps. Our approaches span from devising diagnostics, and therapeutics, and developing disease models to ensure maximal therapeutic benefit is achieved, whilst carefully navigating obstacles related to the research of rare cardiovascular diseases.

Therapeutic Development Services

Diverse Platforms

• Small Molecule Drug Development
• Gene Therapy Development
• Therapeutic Peptide Development

• Cell Therapy Development
• Therapeutic Antibody Development
• Therapeutic Protein Development

Animal Model Development Services

The usage of animal models is of paramount importance in the development of new drugs as they help understand the underlying factors of disease, evaluate the therapy's efficacy along with its safety, and determine adverse consequences that might be presented before human tests are conducted. At our company, we focus on the development of animal models of restrictive cardiomyopathy to aid in the economic shift toward the application of novel innovative therapies.

Genetically Engineered Models

• Dcbld2 knockout model
• P209L overexpression model
• R405W knock-in model

• Q526X knock-in model
• RLC-47K mutant model
• Other models

By our philosophy of sustainable development, Protheragen's team works to achieve the highest standards of quality, compliance, and scientific rigor. We offer complete pharmacokinetics and drug safety evaluation services for the development of new therapeutic options for restrictive cardiomyopathy and other ailments. Feel free to reach out to us for any queries, and rest assured we will provide you the more information and a detailed quote within the shortest possible time.

References

• Chintanaphol, Michelle et al. "Restrictive cardiomyopathy: from genetics and clinical overview to animal modeling." Reviews in cardiovascular medicine 23.3 (2022): 108.
• Rapezzi, Claudio et al. "Restrictive cardiomyopathy: definition and diagnosis." European heart journal 43.45 (2022): 4679-4693.