Elodie Vimont
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View article: Myocardial disarray drives metabolic inefficiency in human cardiomyocytes
Myocardial disarray drives metabolic inefficiency in human cardiomyocytes Open
Adult cardiomyocytes are embedded within a highly organized myocardial microenvironment that imposes critical geometric cues essential for the alignment and distribution of organelles and the shaping of their unique, rectangular cellular m…
View article: Mutant CHCHD10 disrupts cytochrome <i>c</i> oxidation and activates mitochondrial retrograde signaling in a model of cardiomyopathy
Mutant CHCHD10 disrupts cytochrome <i>c</i> oxidation and activates mitochondrial retrograde signaling in a model of cardiomyopathy Open
Mutations in CHCHD10 , a mitochondrial intermembrane space (IMS) protein implicated in proteostasis and cristae maintenance, cause multi-systemic mitochondrial disease. Heterozygous Chchd10 knock-in mice modeling the human CHCHD10 S59L var…
View article: Mtfp1 ablation enhances mitochondrial respiration and protects against hepatic steatosis
Mtfp1 ablation enhances mitochondrial respiration and protects against hepatic steatosis Open
View article: <i>Mtfp1</i>ablation enhances mitochondrial respiration and protects against hepatic steatosis
<i>Mtfp1</i>ablation enhances mitochondrial respiration and protects against hepatic steatosis Open
Hepatic steatosis is the result of an imbalance between nutrient delivery and metabolism in the liver. It is the first hallmark of Non-alcoholic fatty liver disease (NAFLD) and is characterized by the accumulation of excess lipids in the l…
View article: Mitochondrial Fission Process 1 controls inner membrane integrity and protects against heart failure
Mitochondrial Fission Process 1 controls inner membrane integrity and protects against heart failure Open
Mitochondria are paramount to the metabolism and survival of cardiomyocytes. Here we show that Mitochondrial Fission Process 1 (MTFP1) is an inner mitochondrial membrane (IMM) protein that is dispensable for mitochondrial division yet esse…
View article: Mitochondrial fission process 1 (MTFP1) controls bioenergetic efficiency and prevents inflammatory cardiomyopathy and heart failure in mice
Mitochondrial fission process 1 (MTFP1) controls bioenergetic efficiency and prevents inflammatory cardiomyopathy and heart failure in mice Open
Mitochondria are paramount to the metabolism and survival of cardiomyocytes. Here we show that Mitochondrial Fission Process 1 (MTFP1) is essential for cardiac structure and function. Constitutive knockout of cardiomyocyte MTFP1 in mice re…
View article: High‐throughput screening identifies suppressors of mitochondrial fragmentation in <i>OPA1</i> fibroblasts
High‐throughput screening identifies suppressors of mitochondrial fragmentation in <i>OPA1</i> fibroblasts Open
View article: High-throughput phenotypic screen for genetic modifiers in patient-derived<i>OPA1</i>mutant fibroblasts identifies<i>PGS1</i>as a functional suppressor of mitochondrial fragmentation
High-throughput phenotypic screen for genetic modifiers in patient-derived<i>OPA1</i>mutant fibroblasts identifies<i>PGS1</i>as a functional suppressor of mitochondrial fragmentation Open
Mutations affecting the mitochondrial fusion protein Optic Atrophy 1 (OPA1) cause autosomal dominant optic atrophy (DOA) – one of the most common form of mitochondrial disease. The majority of patients develop isolated optic atrophy, but a…