Novel rat model of multiple mitochondrial dysfunction syndromes (MMDS) complicated with cardiomyopathy.

ABSTRACT:

Background

Multiple mitochondrial dysfunction syndromes (MMDS) presents as complex mitochondrial damage, thus impairing a variety of metabolic pathways. Heart dysplasia has been reported in MMDS patients; however, the specific clinical symptoms and pathogenesis remain unclear. More urgently, there is a lack of an animal model to aid research. Therefore, we selected a reported MMDS causal gene, Isca1, and established an animal model of MMDS complicated with cardiac dysplasia.

Methods

The myocardium-specific Isca1 knockout heterozygote (Isca1 HET) rat was obtained by crossing the Isca1 conditional knockout (Isca1 cKO) rat with the α myosin heavy chain Cre (α-MHC-Cre) rat. Cardiac development characteristics were determined by EC

SUBMITTER: Ling Y 

PROVIDER: S-EPMC8690978 | biostudies-literature | 2021 Dec

REPOSITORIES: biostudies-literature