Ontology highlight
ABSTRACT:
SUBMITTER: Mishra P
PROVIDER: S-EPMC7853990 | biostudies-literature | 2019 Dec
REPOSITORIES: biostudies-literature
Mishra Prashant P Zhang Ting T Guo Ming M Chan David D
Bio-protocol 20191205 23
Mitochondrial dysfunction is associated with a number of human diseases. As an example, we recently established <i>in vivo Drosophila</i> models of IBMPFD (Inclusion body myopathy, Paget disease, and frontotemporal dementia), and uncovered that human disease mutations of the <i>p97/VCP</i> (Valosin Containing Protein) gene behave as hyperactive alleles associated with mitochondrial defects. Pharmacologic inhibition of <i>VCP</i> strongly suppressed disease and mitochondrial pathology in these an ...[more]