Search

Your search keyword '"Bernatchez, Pascal"' showing total 31 results
Start Over Author "Bernatchez, Pascal" Publisher wiley
31 results on '"Bernatchez, Pascal"'

1. Magnetic resonance quantification of skeletal muscle lipid infiltration in a humanized mouse model of Duchenne muscular dystrophy

Author

Khattri, Ram B., primary, Batra, Abhinandan, additional, Matheny, Michael, additional, Hart, Cora, additional, Henley‐Beasley, Spencer C., additional, Hammers, David, additional, Zeng, Huadong, additional, White, Zoe, additional, Ryan, Terence E., additional, Barton, Elisabeth, additional, Bernatchez, Pascal, additional, and Walter, Glenn A., additional

Published
2022
Full Text
View/download PDF

30. Magnetic resonance quantification of skeletal muscle lipid infiltration in a humanized mouse model of Duchenne muscular dystrophy.

Author

Khattri RB, Batra A, Matheny M, Hart C, Henley-Beasley SC, Hammers D, Zeng H, White Z, Ryan TE, Barton E, Bernatchez P, and Walter GA

Subjects
Animals, Mice, Mice, Inbred mdx, Muscle, Skeletal diagnostic imaging, Muscle, Skeletal pathology, Magnetic Resonance Imaging, Magnetic Resonance Spectroscopy, Cholesterol, Apolipoproteins E, Disease Models, Animal, Muscular Dystrophy, Duchenne diagnostic imaging, Muscular Dystrophy, Duchenne pathology
Abstract

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 deposition in two preclinical models of muscular dystrophy (Dysferlin-null and mdx mice) through apolipoprotein E (ApoE) gene deletion without and with cholesterol-, triglyceride-rich Western diet supplementation, we sought to determine whether magnetic resonance imaging and spectroscopy (MRI and MRS, respectively) could be used to detect, characterize, and compare lipid deposition in mdx-ApoE knockout with mdx mice in a diet-dependent manner. MRI revealed that both mdx and mdx-ApoE mice exhibited elevated proton relaxation time constants (T 2 ) in their lower hindlimbs irrespective of diet, indicating both chronic muscle damage and fatty tissue deposition. The mdx-ApoE mice on a Western diet (mdx-ApoE W ) presented with greatest fatty tissue infiltration in the posterior compartment of the hindlimb compared with other groups, as detected by MRI/MRS. High-resolution magic angle spinning confirmed elevated lipid deposition in the posterior compartments of mdx-ApoE W mice in vivo and ex vivo, respectively. In conclusion, the mdx-ApoE W model recapitulates some of the extreme fatty tissue deposition observed clinically in DMD muscle but typically absent in mdx mice. This preclinical model will help facilitate the development of new imaging modalities directly relevant to the image contrast generated in DMD, and help to refine MR-based biomarkers and their relationship to tissue structure and disease progression., (© 2022 John Wiley & Sons Ltd.)

Published
2023
Full Text
View/download PDF

31. Dysferlin deficiency confers increased susceptibility to coxsackievirus-induced cardiomyopathy.

Author

Wang C, Wong J, Fung G, Shi J, Deng H, Zhang J, Bernatchez P, and Luo H

Subjects
Animals, Dysferlin, Mice, Coxsackievirus Infections genetics, Coxsackievirus Infections pathology, Genetic Predisposition to Disease, Membrane Proteins deficiency, Myocarditis genetics, Myocarditis pathology
Abstract

Coxsackievirus infection can lead to viral myocarditis and its sequela, dilated cardiomyopathy, which represent major causes of cardiovascular mortality worldwide in children. Yet, the host genetic susceptible factors and the underlying mechanisms by which viral infection damages cardiac function remain to be fully resolved. Dysferlin is a transmembrane protein highly expressed in skeletal and cardiac muscles. In humans, mutations in the dysferlin gene can cause limb-girdle muscular dystrophy type 2B and Miyoshi myopathy. Dysferlin deficiency has also been linked to cardiomyopathy. Defective muscle membrane repair has been suggested to be an important mechanism responsible for muscle degeneration in dysferlin-deficient patients and animals. Using both naturally occurring and genetically engineered dysferlin-deficient mice, we demonstrated that loss of dysferlin confers increased susceptibility to coxsackievirus infection and myocardial damage. More interestingly, we found that dysferlin is cleaved following coxsackieviral infection through the proteolytic activity of virally encoded proteinases, suggesting an important mechanism underlying virus-induced cardiac dysfunction. Our results in this study not only identify dysferlin deficiency as a novel host risk factor for viral myocarditis but also reveal a key mechanism by which coxsackievirus infection impairs cardiac function, leading to the development of dilated cardiomyopathy., (© 2015 John Wiley & Sons Ltd.)

Published
2015
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results