The newborn Fmr1 knockout mouse: a novel model of excess ubiquinone and closed mitochondrial permeability transition pore in the developing heart.

CONCLUSION: These findings establish the newborn Fmr1 KO mouse as a novel model of excess ubiquinone and closed mPTP in the developing heart. Such a model may help provide insight into the biology of cardiac development and pathophysiology of neonatal heart failure.
IMPACT: Ubiquinone is in excess and the mPTP is closed in the developing FXS heart.Strengthens evidence of open mPTP probability in the normally developing postnatal murine heart and provides new evidence for premature closure of the mPTP in Fmr1 mutants.Establishes a novel model of excess CoQ and a closed pore in the developing heart. Such a model will be a valuable tool used to better understand the role of ubiquinone and the mPTP in the neonatal heart in health and disease.
PMID: 32674111 [PubMed - as supplied by publisher]

Source: Pediatric Research - July 15, 2020 Category: Pediatrics Authors: Barajas M, Wang A, Griffiths KK, Matsumoto K, Liu R, Homma S, Levy RJ Tags: Pediatr Res Source Type: research