Pascal Bernatchez
YOU?
Author Swipe
View article: Exploring Pulse Wave Velocity as a Vascular Hemodynamic Stress Marker: More Than Just Arterial Stiffening?
Exploring Pulse Wave Velocity as a Vascular Hemodynamic Stress Marker: More Than Just Arterial Stiffening? Open
Arterial pulse wave velocity (PWV), defined as the speed at which a blood pressure pulse propagates along the arterial tree, is the gold standard for assessment of arterial stiffness and can serve as an independent predictor of cardiovascu…
View article: High‐Density Lipoprotein‐Associated Cholesterol Abnormalities in a Clinical Outcomes Study of Dysferlin‐Deficient Limb–Girdle Muscular Dystrophy Type R2
High‐Density Lipoprotein‐Associated Cholesterol Abnormalities in a Clinical Outcomes Study of Dysferlin‐Deficient Limb–Girdle Muscular Dystrophy Type R2 Open
Background Limb–girdle muscular dystrophy (MD) type R2 (LGMDR2, formerly LGMD2B) is an autosomal recessive form of MD caused by variants in the dysferlin gene, DYSF . It leads to slow proximal and distal muscle weakening that generally res…
View article: Scaffolds and the scaffolding domain: an alternative paradigm for caveolin-1 signaling
Scaffolds and the scaffolding domain: an alternative paradigm for caveolin-1 signaling Open
Caveolin-1 (Cav1) is a 22 kDa intracellular protein that is the main protein constituent of bulb-shaped membrane invaginations known as caveolae. Cav1 can be also found in functional non-caveolar structures at the plasma membrane called sc…
View article: Humanization of the mdx Mouse Phenotype for Duchenne Muscular Dystrophy Modeling: A Metabolic Perspective
Humanization of the mdx Mouse Phenotype for Duchenne Muscular Dystrophy Modeling: A Metabolic Perspective Open
Duchenne muscular dystrophy (DMD) is a severe form of muscular dystrophy (MD) that is characterized by early muscle wasting and lethal cardiorespiratory failure. While the mdx mouse is the most common model of DMD, it fails to replicate th…
View article: Dyslipidemia in Muscular Dystrophy: A Systematic Review and Meta-Analysis
Dyslipidemia in Muscular Dystrophy: A Systematic Review and Meta-Analysis Open
Background: Muscular dystrophies (MDs) are characterized by chronic muscle wasting but also poorly understood metabolic co-morbidities. We have recently shown that Duchenne MD (DMD) patients, dogs and asymptomatic carriers are affected by …
View article: Limb-girdle muscular dystrophy type 2B causes HDL-C abnormalities in patients and statin-resistant muscle wasting in dysferlin-deficient mice
Limb-girdle muscular dystrophy type 2B causes HDL-C abnormalities in patients and statin-resistant muscle wasting in dysferlin-deficient mice Open
Limb-girdle muscular dystrophy (MD) type 2B (LGMD2B) and Duchenne MD (DMD) are caused by mutations to the Dysferlin and Dystrophin genes, respectively. We have recently demonstrated in typically mild dysferlin- and dystrophin-deficient mou…
View article: Magnetic resonance quantification of skeletal muscle lipid infiltration in a humanized mouse model of Duchenne muscular dystrophy
Magnetic resonance quantification of skeletal muscle lipid infiltration in a humanized mouse model of Duchenne muscular dystrophy Open
Rodent models of Duchenne muscular dystrophy (DMD) often do not recapitulate the severity of muscle wasting and resultant fibro‐fatty infiltration observed in DMD patients. Having recently documented severe muscle wasting and fatty deposit…
View article: Lipid nanoparticle formulations for optimal RNA-based topical delivery to murine airways
Lipid nanoparticle formulations for optimal RNA-based topical delivery to murine airways Open
LNP formulations enable the delivery of RNA payloads into human airway epithelial cells, and in the murine respiratory system; they can be delivered to nasal mucosa and lower respiratory tract via intranasal delivery. The composition of he…
View article: A Role of Caveolae in Trabecular Meshwork Mechanosensing and Contractile Tone
A Role of Caveolae in Trabecular Meshwork Mechanosensing and Contractile Tone Open
Polymorphisms in the CAV1/2 gene loci impart increased risk for primary open-angle glaucoma (POAG). CAV1 encodes caveolin-1 (Cav1), which is required for biosynthesis of plasma membrane invaginations called caveolae. Cav1 knockout mice exh…
View article: Cholesterol absorption blocker ezetimibe prevents muscle wasting in severe dysferlin‐deficient and <i>mdx</i> mice
Cholesterol absorption blocker ezetimibe prevents muscle wasting in severe dysferlin‐deficient and <i>mdx</i> mice Open
Background Muscular dystrophy (MD) causes muscle wasting and is often lethal in patients due to a lack of proven therapies. In contrast, mouse models of MD are notoriously mild. We have previously shown severe human‐like muscle pathology i…
View article: Effect of Dysferlin Deficiency on Atherosclerosis and Plasma Lipoprotein Composition Under Normal and Hyperlipidemic Conditions
Effect of Dysferlin Deficiency on Atherosclerosis and Plasma Lipoprotein Composition Under Normal and Hyperlipidemic Conditions Open
Dysferlinopathies are a group of muscle disorders caused by mutations to dysferlin, a transmembrane protein involved in membrane patching events following physical damage to skeletal myofibers. We documented dysferlin expression in vascula…
View article: Characterization of a Novel Caveolin Modulator That Reduces Vascular Permeability and Ocular Inflammation
Characterization of a Novel Caveolin Modulator That Reduces Vascular Permeability and Ocular Inflammation Open
CVX51401 is an optimized Cav modulator that reduces vascular permeability and ocular inflammation that is poised for clinical development.
View article: Blood pressure‐independent inhibition of Marfan aortic root widening by the angiotensin II receptor blocker valsartan
Blood pressure‐independent inhibition of Marfan aortic root widening by the angiotensin II receptor blocker valsartan Open
Marfan syndrome (MFS) is a genetic disorder that results in accelerated aortic root widening and aneurysm. However, management of MFS patients with blood pressure (BP)-lowering medications, such as angiotensin II (AngII) receptor blocker (…
View article: Radiofrequency Therapy Improves Exercise Capacity of Mice with Emphysema
Radiofrequency Therapy Improves Exercise Capacity of Mice with Emphysema Open
Background; Emphysema is a common phenotype of chronic obstructive pulmonary disease (COPD). Although resection of emphysematous tissue can improve lung mechanics, it is invasive and fraught with adverse effects. Meanwhile, radiofrequency …
View article: Exercise during pregnancy mitigates the adverse effects of maternal obesity on adult male offspring vascular function and alters one‐carbon metabolism
Exercise during pregnancy mitigates the adverse effects of maternal obesity on adult male offspring vascular function and alters one‐carbon metabolism Open
Maternal obesity during pregnancy can adversely affect adult offspring vascular endothelial function. This study examined whether maternal exercise during pregnancy and lactation mitigates the adverse effects of maternal obesity on offspri…
View article: Characterization of doxycycline-mediated inhibition of Marfan syndrome-associated aortic dilation by multiphoton microscopy
Characterization of doxycycline-mediated inhibition of Marfan syndrome-associated aortic dilation by multiphoton microscopy Open
Marfan syndrome (MFS) is a connective tissue disorder that results in aortic root widening and aneurysm if unmanaged. We have previously reported doxycycline, a nonselective matrix metalloproteinases (MMPs) inhibitor, to attenuate aortic r…
View article: Proceedings of the Ninth HDL (High-Density Lipoprotein) Workshop
Proceedings of the Ninth HDL (High-Density Lipoprotein) Workshop Open
The HDL (high-density lipoprotein) Workshop was established in 2009 as a forum for candid discussions among academic basic scientists, clinical investigators, and industry researchers about the role of HDL in cardiovascular disease. This n…
View article: Angiotensin II receptor blocker losartan exacerbates muscle damage and exhibits weak blood pressure-lowering activity in a dysferlin-null model of Limb-Girdle muscular dystrophy type 2B
Angiotensin II receptor blocker losartan exacerbates muscle damage and exhibits weak blood pressure-lowering activity in a dysferlin-null model of Limb-Girdle muscular dystrophy type 2B Open
There is no cure or beneficial management option for Limb-Girdle muscular dystrophy (MD) type 2B (LGMD2B). Losartan, a blood pressure (BP) lowering angiotensin II (AngII) receptor type 1 (ATR1) blocker (ARB) with unique anti-transforming g…
View article: Podocalyxin is required for maintaining blood–brain barrier function during acute inflammation
Podocalyxin is required for maintaining blood–brain barrier function during acute inflammation Open
Significance This article demonstrates a role for podocalyxin in blood–brain barrier (BBB) function. Podocalyxin expression on endothelial cells promotes the formation of adhesion interactions and barrier formation. Furthermore, it describ…
View article: In vivo characterization of doxycycline-mediated protection of aortic function and structure in a mouse model of Marfan syndrome-associated aortic aneurysm
In vivo characterization of doxycycline-mediated protection of aortic function and structure in a mouse model of Marfan syndrome-associated aortic aneurysm Open
Aortic aneurysm is the most life-threatening complication in Marfan syndrome (MFS) patients. Doxycycline, a nonselective matrix metalloproteinases inhibitor, was reported to improve the contractile function and elastic fiber structure and …