Lumican accumulates with fibrillar collagen in fibrosis in hypertrophic cardiomyopathy.

ABSTRACT:

Aims

Familial hypertrophic cardiomyopathy (HCM) is the most common form of inherited cardiac disease. It is characterized by myocardial hypertrophy and diastolic dysfunction, and can lead to severe heart failure, arrhythmias, and sudden cardiac death. Cardiac fibrosis, defined by excessive accumulation of extracellular matrix (ECM) components, is central to the pathophysiology of HCM. The ECM proteoglycan lumican is increased during heart failure and cardiac fibrosis, including HCM, yet its role in HCM remains unknown. We provide an in-depth assessment of lumican in clinical and experimental HCM.

Methods

Left ventricular (LV) myectomy specimens were collected from patients with hypertrophic obstructive cardiomyopathy (n = 15), and controls from hearts deemed unsuitable for t

SUBMITTER: Rixon C 

PROVIDER: S-EPMC10053290 | biostudies-literature | 2023 Apr

REPOSITORIES: biostudies-literature