Ontology highlight
ABSTRACT:
SUBMITTER: Kustermann M
PROVIDER: S-EPMC6152520 | biostudies-literature | 2018
REPOSITORIES: biostudies-literature
Kustermann Monika M Manta Linda L Paone Christoph C Kustermann Jochen J Lausser Ludwig L Wiesner Cora C Eichinger Ludwig L Clemen Christoph S CS Schröder Rolf R Kestler Hans A HA Sandri Marco M Rottbauer Wolfgang W Just Steffen S
Autophagy 20180816 11
VCP/p97 (valosin containing protein) is a key regulator of cellular proteostasis. It orchestrates protein turnover and quality control in vivo, processes fundamental for proper cell function. In humans, mutations in VCP lead to severe myo- and neuro-degenerative disorders such as inclusion body myopathy with Paget disease of the bone and frontotemporal dementia (IBMPFD), amyotrophic lateral sclerosis (ALS) or and hereditary spastic paraplegia (HSP). We analyzed here the in vivo role of Vcp and i ...[more]