Animal models of Duchenne muscular dystrophy: from basic mechanisms to gene therapy
JW McGreevy, CH Hakim… - Disease models & …, 2015 - journals.biologists.com
Duchenne muscular dystrophy (DMD) is a progressive muscle-wasting disorder. It is caused
by loss-of-function mutations in the dystrophin gene. Currently, there is no cure. A highly …
by loss-of-function mutations in the dystrophin gene. Currently, there is no cure. A highly …
Extracellular matrix: the driving force of mammalian diseases
RV Iozzo, MA Gubbiotti - Matrix Biology, 2018 - Elsevier
Like the major theme of a Mozart concerto, the immense and pervasive extracellular matrix
drives each movement and ultimately closes the symphony, embracing a unique role as the …
drives each movement and ultimately closes the symphony, embracing a unique role as the …
Zebrafish: an emerging model system for human disease and drug discovery
In vivo studies represent an essential step in drug development and currently rely largely on
mice, yet limitations of mammalian models motivated the search for complementary …
mice, yet limitations of mammalian models motivated the search for complementary …
[HTML][HTML] Efficient and Reproducible Myogenic Differentiation from Human iPS Cells: Prospects for Modeling Miyoshi Myopathy In Vitro
The establishment of human induced pluripotent stem cells (hiPSCs) has enabled the
production of in vitro, patient-specific cell models of human disease. In vitro recreation of …
production of in vitro, patient-specific cell models of human disease. In vitro recreation of …
[HTML][HTML] MicroRNA-486–dependent modulation of DOCK3/PTEN/AKT signaling pathways improves muscular dystrophy–associated symptoms
Duchenne muscular dystrophy (DMD) is caused by mutations in the gene encoding
dystrophin, which results in dysfunctional signaling pathways within muscle. Previously, we …
dystrophin, which results in dysfunctional signaling pathways within muscle. Previously, we …
miRNAS in normal and diseased skeletal muscle
I Eisenberg, MS Alexander… - Journal of cellular and …, 2009 - Wiley Online Library
• Introduction• Mechanisms of miRNA‐mediated repression• miRNA regulatory network in
muscle‐Muscle‐specific miRNAs‐Non‐muscle‐specific miRNAs• miRNA signatures as …
muscle‐Muscle‐specific miRNAs‐Non‐muscle‐specific miRNAs• miRNA signatures as …
A central function for perlecan in skeletal muscle and cardiovascular development
JJ Zoeller, A McQuillan, J Whitelock, SY Ho… - The Journal of cell …, 2008 - rupress.org
Perlecan's developmental functions are difficult to dissect in placental animals because
perlecan disruption is embryonic lethal. In contrast to mammals, cardiovascular function is …
perlecan disruption is embryonic lethal. In contrast to mammals, cardiovascular function is …
Zebrafish relatively relaxed mutants have a ryanodine receptor defect, show slow swimming and provide a model of multi-minicore disease
H Hirata, T Watanabe, J Hatakeyama, SM Sprague… - 2007 - journals.biologists.com
Wild-type zebrafish embryos swim away in response to tactile stimulation. By contrast,
relatively relaxed mutants swim slowly due to weak contractions of trunk muscles …
relatively relaxed mutants swim slowly due to weak contractions of trunk muscles …
[HTML][HTML] Model Organisms in the Fight against Muscular Dystrophy: Lessons from Drosophila and Zebrafish
E Plantié, M Migocka-Patrzałek, M Daczewska, K Jagla - Molecules, 2015 - mdpi.com
Muscular dystrophies (MD) are a heterogeneous group of genetic disorders that cause
muscle weakness, abnormal contractions and muscle wasting, often leading to premature …
muscle weakness, abnormal contractions and muscle wasting, often leading to premature …
Cell-lineage regulated myogenesis for dystrophin replacement: a novel therapeutic approach for treatment of muscular dystrophy
E Kimura, JJ Han, S Li, B Fall, J Ra… - Human molecular …, 2008 - academic.oup.com
Duchenne muscular dystrophy (DMD) is characterized in skeletal muscle by cycles of
myofiber necrosis and regeneration leading to loss of muscle fibers and replacement with …
myofiber necrosis and regeneration leading to loss of muscle fibers and replacement with …