Target intelligence / Profile preview

Plakophilin 2 (PKP2)

Target: PKP2
Molecular classification: Armadillo repeat-containing protein, Plakophilin family

Overview

Plakophilin 2 (PKP2) is a protein crucial for cell-cell adhesion, particularly in cardiac muscle and skin. As a key component of desmosomes, it links cadherins to intermediate filaments, providing mechanical strength to tissues under stress. Mutations in PKP2 are strongly associated with Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), leading to impaired desmosome function, arrhythmias, and increased risk of sudden death. PKP2 also plays a role in mitochondrial function, nuclear envelope integrity, and regulation of transcription.

Other names: PKP2 protein

Biological functions

Cell-cell adhesionDesmosome assemblyRegulation of RhoA signalingMitochondrial functionNuclear envelope integrityRegulation of transcription

Disease associations

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

Safety considerations

Potential for arrhythmias (ARVC)Sudden death (ARVC)

Biomarkers

PKP2 mutations (ARVC)

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