A5006223586- Muscle Physiology and Disorders
- Adipose Tissue and Metabolism
- Cardiomyopathy and Myosin Studies
- Mitochondrial Function and Pathology
- Neurogenetic and Muscular Disorders Research
- Silk-based biomaterials and applications
- Sirtuins and Resveratrol in Medicine
- Genetic Neurodegenerative Diseases
- Circadian rhythm and melatonin
- Microgrid Control and Optimization
- Congenital Anomalies and Fetal Surgery
- Energy Harvesting in Wireless Networks
- Photoreceptor and optogenetics research
- Muscle metabolism and nutrition
- Electrospun Nanofibers in Biomedical Applications
- RNA Research and Splicing
- Sleep and Wakefulness Research
- RNA modifications and cancer
- Kruppel-like factors research
University of Oxford
2015-2020
Leiden University Medical Center
2014
Significance The inhibitory neurotransmitter GABA plays an important role in many brain circuits involved anxiety, depression, epilepsy, and sleep disorders. is critical for maintaining the balance between excitatory transmission, thereby ensuring proper neuronal network function. Here, we report that entraining mice to a long-day photoperiod can modify excitatory/inhibitory suprachiasmatic by increasing GABAergic excitation. These data suggest day length affects of circadian clock. This...
The circadian glucocorticoid-Krüppel-like factor 15-branched-chain amino acid (GC-KLF15-BCAA) signaling pathway is a key regulatory axis in muscle, whose imbalance has wide-reaching effects on metabolic homeostasis. Spinal muscular atrophy (SMA) neuromuscular disorder also characterized by intrinsic muscle pathologies, abnormalities and disrupted sleep patterns, which can influence or be influenced networks that control behavioral rhythms. We therefore set out to investigate the contribution...
The absence of the dystrophin protein in Duchenne muscular dystrophy (DMD) results myofiber fragility and a plethora downstream secondary pathologies. Although variety experimental therapies are development, achieving effective treatments for DMD remains exceptionally challenging, not least because pathological consequences loss incompletely understood. Here we have performed proteome profiling tibialis anterior muscles from two murine models (
Abstract Background Duchenne muscular dystrophy (DMD) is a fatal muscle‐wasting disorder caused by genetic loss of dystrophin protein. Extracellular microRNAs (ex‐miRNAs) are putative, minimally invasive biomarkers DMD. Specific ex‐miRNAs (e.g. miR‐1, miR‐133a, miR‐206, and miR‐483) highly up‐regulated in the serum DMD patients dystrophic animal models restored to wild‐type levels following exon skipping‐mediated rescue mdx mice. As such, promising pharmacodynamic skipping efficacy. Here, we...
Physiology and behaviour are critically dependent on circadian regulation via a core set of clock genes, dysregulation which leads to metabolic sleep disturbances. Metabolic perturbations occur in spinal muscular atrophy (SMA), neuromuscular disorder caused by loss the survival motor neuron (SMN) protein characterized muscle atrophy. We therefore investigated expression rhythm genes various tissues cord Taiwanese Smn-/-;SMN2 SMA animal model. demonstrate dysregulated (clock, ARNTL/Bmal1,...
Duchenne muscular dystrophy (DMD) is caused by loss of dystrophin protein, leading to progressive muscle weakness and premature death due respiratory and/or cardiac complications. Cardiac involvement characterized dilated cardiomyopathy, decreased fractional shortening metabolic dysfunction involving reduced metabolism fatty acids-the major substrate. Several mouse models have been developed study molecular pathological consequences deficiency, but do not recapitulate all aspects human...