Open AccessCombined therapy with PPARα agonist and l-carnitine rescues lipotoxic cardiomyopathy due to systemic carnitine deficiency
Author(s) -
T ASAI,
Kenji Okumura,
R TAKAHASHI,
Hiroyuki Matsui,
Y. Numaguchi,
Hisashi Murakami,
Ryuichiro Murakami,
T MUROHARA
Publication year - 2006
Publication title -
cardiovascular research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.774
H-Index - 219
eISSN - 1755-3245
pISSN - 0008-6363
DOI - 10.1016/j.cardiores.2006.02.005
Subject(s) - carnitine , cardiomyopathy , medicine , agonist , endocrinology , cardiology , heart failure , receptor
Peroxisome proliferator-activated receptors (PPAR) are ligand-activated transcription factors that belong to the nuclear hormone receptor superfamily and are key regulators of fatty acid oxidation (FAO) in the heart. Systemic carnitine deficiency (SCD) causes disorders of FAO and induces hypertrophic cardiomyopathy with lipid accumulation. We hypothesized that activation of PPARalpha by fenofibrate, a PPARalpha agonist, in addition to conventional L-carnitine supplementation may exert beneficial effects on the lipotoxic cardiomyopathy in juvenile visceral steatosis (JVS) mouse, a murine model of SCD.
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