Thursday, March 28, 2013
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Models for pathogenic
mutations in vasolin containing protein
Fruit flies and human cells
with pathogenic mutations in VCP could be useful as models to study diseases
associated with such mutations, such as frontotemporal dementia (FTD) and
amyotrophic lateral sclerosis (ALS). In patient fibroblasts or human CNS cell
lines, mutant VCP decreased mitochondrial membrane potential and increased
mitochondrial respiration compared with wild-type VCP. Fruit flies with
pathogenic mutations in the VCP ortholog showed defects in neuromuscular
junction morphology, muscle degeneration and other myopathy. Next steps could
include using the models to evaluate therapeutic candidates.
Published online March 28, 2013
Patent and licensing status
unavailable for both studies
Bartolome, F. et al.
Neuron; published online March 14, 2013;
Contact: Helene Plun-Favreau, UCL Institute of Neurology, London,
Contact: Andrey Y. Abramov, same affiliation as above
Kim, N.C. et al. Neuron; published online March 14, 2013;
Contact: J. Paul Taylor, St. Jude Children's Research Hospital,
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