Impaired Reorganization of Centrosome Structure Underlies Human Infantile Dilated Cardiomyopathy.

ABSTRACT:

Background

During cardiomyocyte maturation, the centrosome, which functions as a microtubule organizing center in cardiomyocytes, undergoes dramatic structural reorganization where its components reorganize from being localized at the centriole to the nuclear envelope. This developmentally programmed process, referred to as centrosome reduction, has been previously associated with cell cycle exit. However, understanding of how this process influences cardiomyocyte cell biology, and whether its disruption results in human cardiac disease, remains unknown. We studied this phenomenon in an infant with a rare case of infantile dilated cardiomyopathy (iDCM) who presented with left ventricular ejection fraction of 18% and disrupted sarcomere and mitochondria structure.

Methods

We

SUBMITTER: Chun YW 

PROVIDER: S-EPMC10133173 | biostudies-literature | 2023 Apr

REPOSITORIES: biostudies-literature