Rapamycin and chloroquine: the in vitro and in vivo effects of autophagy-modifying drugs show promising results in valosin containing protein multisystem proteinopathy

Mutations in the valosin containing protein (VCP) gene cause hereditary Inclusion body myopathy (hIBM) associated with Paget disease of bone (PDB), frontotemporal dementia (FTD), more recently termed multisystem proteinopathy (MSP). Affected individuals exhibit scapular winging and die from progress...

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Veröffentlicht in:	PloS one 2015-04, Vol.10 (4), p.e0122888-e0122888
Hauptverfasser:	Nalbandian, Angèle, Llewellyn, Katrina J, Nguyen, Christopher, Yazdi, Puya G, Kimonis, Virginia E
Format:	Artikel
Sprache:	eng
Schlagworte:	Accumulation Amyotrophic lateral sclerosis Analysis Animals Apoptosis Apoptosis - drug effects Autophagy - drug effects Cell Cycle Proteins Cell death Cell Line Chloroquine Chloroquine - pharmacology Defects Dementia disorders Deoxyribonucleic acid Disease Models, Animal Disorders DNA DNA-binding protein DNA-Binding Proteins - metabolism Drugs Eye Proteins - metabolism Frontotemporal dementia Frontotemporal Dementia - metabolism Frontotemporal Dementia - pathology Gene Knock-In Techniques Heart diseases Humans Inclusion bodies Inhibitors Intercellular Signaling Peptides and Proteins Kinases Lysosomes Male Membrane Transport Proteins Mice Muscle, Skeletal - metabolism Muscle, Skeletal - pathology Muscles Mutation Myoblasts Myoblasts - cytology Myoblasts - drug effects Myoblasts - metabolism Myopathy Osteitis Deformans - metabolism Osteitis Deformans - pathology Pathology Patients Peptides - genetics Peptides - metabolism Phagocytosis Pharmacology Protein binding Quadriceps muscle Rapamycin Rodents Signal transduction Signal Transduction - drug effects Signaling Sirolimus - pharmacology Substrate inhibition Substrates TOR protein TOR Serine-Threonine Kinases - metabolism Ubiquitin Ubiquitin - metabolism Vacuoles
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