Progressive Cardiac Conduction Defect (PCCD)

Progressive cardiac conduction defect (PCCD) is a grave disorder of the heart characterized by prolonged impulse propagation. PCCD evolves with time and has an age-dependent penetrance. Protheragen is a groundbreaking research service provider focused on advancing the understanding of rare cardiovascular diseases, including PCCD. Our custom-tailored services in comprehensive drug research and development stand at the forefront of serving client needs.

Overview of Progressive Cardiac Conduction Defect (PCCD)

Progressive cardiac conduction defect (PCCD) which is sometimes referred to as Lev-Lenegre disease, is a form of genetic heart disease that causes the progressing delay of impulse conduction in the His-Purkinje system with right or left bundle branch block (RBBB or LBBB), susceptibility to atrioventricular (AV) block, syncope and in some cases sudden cardiac death (SCD). The term PCCD includes forms of the disorder with disease either congenital or acquired, with or without other forms of structural heart disease.

Fig.1 Main mechanisms involved in the pathogenesis of PCCD. (Asatryan, B., and Medeiros-Domingo, A., 2019)

Pathogenesis of Progressive Cardiac Conduction Defect (PCCD)

Current knowledge suggests that familial PCCD, in the absence of any structural or congenital heart disease as well as systemic disease, usually stems from mutations within the genes encoding cardiac ion channels which are involved in the conduction of electrical impulses in the heart. On the other hand, PCCD within the context of structural heart disease is considered to stem from mutations in the genes of transcriptional factors, enzymes, or structural proteins. In the past 15 years, the following genes have been reported to have their mutations associated with this disorder: SCNA5, NKX2.5, PRKAG2, LMNA, TRPM4, and GJA5.

Therapeutics Development for Progressive Cardiac Conduction Defect (PCCD)

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

Our Services

With an unwavering commitment to quality and precision, we empower therapy innovations that target some of the most challenging conditions in cardiovascular diseases. Our holistic, sequential platform of drug development and discovery presents complete solutions starting from the development of diagnostics to innovative therapeutics and well-designed disease models.

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 for PCCD

The animal model provides a profound understanding of the genetic and electrophysiological mechanisms for PCCD while presenting new opportunities for drug development. Our company focuses on the design of tailor-made animal model development programs specifically for PCCD drug research to accelerate your research and close the gap from discovery in the laboratory to application.

Genetically Engineering Animal Model

Genetically engineered animal models for PCCD are typically constructed by targeting key genes involved in cardiac conduction and fibrotic remodeling.

Optional models:

• Scn5a^+/- model

• Other models

Moreover, Protheragen provides a wide variety of preclinical services including pharmacokinetics and comprehensive safety analyses. This approach guarantees that every candidate therapeutic is tested for efficacy and safety before advancing to further trials. Count on Protheragen as your partner in developing revolutionary therapies for rare cardiovascular diseases and advancing the future of cardiovascular care. Should you wish to inquire about our services, do not hesitate to reach out to us.

Reference

• Asatryan, Babken, and Argelia Medeiros-Domingo. "Molecular and genetic insights into progressive cardiac conduction disease." Europace: European pacing, arrhythmias, and cardiac electrophysiology: journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology 21.8 (2019): 1145-1158.