Anthony Accorsi
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View article: Optimization of Xenografting Methods for Generating Human Skeletal Muscle in Mice
Optimization of Xenografting Methods for Generating Human Skeletal Muscle in Mice Open
Xenografts of human skeletal muscle generated in mice can be used to study muscle pathology and to test drugs designed to treat myopathies and muscular dystrophies for their efficacy and specificity in human tissue. We previously developed…
View article: p38α Regulates Expression of DUX4 in a Model of Facioscapulohumeral Muscular Dystrophy
p38α Regulates Expression of DUX4 in a Model of Facioscapulohumeral Muscular Dystrophy Open
Facioscapulohumeral muscular dystrophy (FSHD) is caused by the loss of repression at the D4Z4 locus leading to aberrant double homeobox 4 (DUX4) expression in skeletal muscle. Activation of this early embryonic transcription factor results…
View article: Fibrogenesis in LAMA2-Related Muscular Dystrophy Is a Central Tenet of Disease Etiology
Fibrogenesis in LAMA2-Related Muscular Dystrophy Is a Central Tenet of Disease Etiology Open
LAMA2-related congenital muscular dystrophy, also known as MDC1A, is caused by loss-of-function mutations in the alpha2 chain of Laminin-211. Loss of this protein interrupts the connection between the muscle cell and its extracellular envi…
View article: P38α Regulates Expression of DUX4 in Facioscapulohumeral Muscular Dystrophy
P38α Regulates Expression of DUX4 in Facioscapulohumeral Muscular Dystrophy Open
FSHD is caused by the loss of repression at the D4Z4 locus leading to DUX4 expression in skeletal muscle, activation of its early embryonic transcriptional program and muscle fiber death. While progress toward understanding the signals dri…
View article: IGF-1/GH axis enhances losartan treatment in Lama2-related muscular dystrophy
IGF-1/GH axis enhances losartan treatment in Lama2-related muscular dystrophy Open
As the complexities of dystrophic pathology have been elucidated over the last few years, it has become increasingly clear that primary monogenetic defects result in multiple secondary pathologies capable of autonomously driving disease pr…
View article: miR-410 and miR-495 Are Dynamically Regulated in Diverse Cardiomyopathies and Their Inhibition Attenuates Pathological Hypertrophy
miR-410 and miR-495 Are Dynamically Regulated in Diverse Cardiomyopathies and Their Inhibition Attenuates Pathological Hypertrophy Open
Noncoding RNAs have emerged as important modulators in cardiac development and pathological remodeling. Recently, we demonstrated that regulation of the Gtl2-Dio3 noncoding RNA locus is dependent on the MEF2 transcription factor in cardiac…
View article: Magnetic Resonance Imaging Is Sensitive to Pathological Amelioration in a Model for Laminin-Deficient Congenital Muscular Dystrophy (MDC1A)
Magnetic Resonance Imaging Is Sensitive to Pathological Amelioration in a Model for Laminin-Deficient Congenital Muscular Dystrophy (MDC1A) Open
MRI is sensitive to and tightly corresponds with pathological changes in DyW mice and thus is a viable and effective non-invasive tool for assessing pathological changes.
View article: Do's and Don'ts in the Preparation of Muscle Cryosections for Histological Analysis
Do's and Don'ts in the Preparation of Muscle Cryosections for Histological Analysis Open
Histological evaluation of muscle biopsies has served as an indispensable tool in the understanding of the development and progression of pathology of neuromuscular disorders. However, in order to do so, proper care needs to be taken when …
View article: Integrin dysregulation as a possible driver of matrix remodeling in Laminin-deficient congenital muscular dystrophy (MDC1A)
Integrin dysregulation as a possible driver of matrix remodeling in Laminin-deficient congenital muscular dystrophy (MDC1A) Open
Background: Merosin-deficient congenital muscular dystrophy (MDC1A) is caused by a loss of Laminin-α2. Secondary manifestations include failed regeneration, inflammation, and fibrosis; however, specific pathomechanisms remain unknown. Obje…