The ATPase valosin-containing protein (VCP)/p97 has emerged as a central and important
element of the ubiquitin system. Together with a network of cofactors, it regulates an ever-…

Mutations in valosin-containing protein (VCP) cause inclusion body myopathy (IBM) associated
with Paget’s disease of the bone (PDB) and fronto-temporal dementia (FTD) or IBMPFD. …

Neuromuscular disorders comprise a diverse group of human inborn diseases that arise from
defects in the structure and/or function of the muscle tissue — encompassing the muscle …

Algorithms designed to identify canonical yeast prions predict that around 250 human
proteins, including several RNA-binding proteins associated with neurodegenerative disease, …

Mutations in valosin-containing protein (VCP) cause inclusion body myopathy (IBM),
Paget's disease of the bone, and frontotemporal dementia (IBMPFD). Patient muscle has …

MATR3 is an RNA- and DNA-binding protein that interacts with TDP-43, a disease protein
linked to amyotrophic lateral sclerosis (ALS) and frontotemporal dementia. Using exome …

… CC Weihl1, … Dr CC Weihl, Department of Neurology, Washington University School of
Medicine, 660 S Euclid Avenue, St Louis, MO 63110, USA; weihlc{at}neuro.wustl.edu …

Rupture of endosomes and lysosomes is a major cellular stress condition leading to cell
death and degeneration. Here, we identified an essential role for the ubiquitin‐directed AAA ‐ …

… (g) p62-KO and p62-KO/TFEB-TG macrophages were treated with LPS+CC for 24 h or
with LPS followed by ATP for 3 h. Culture media were assayed for IL-1β by ELISA (n=3 …

The AAA-ATPase VCP (also known as p97) cooperates with distinct cofactors to process
ubiquitylated proteins in different cellular pathways 1 , 2 , 3 . VCP missense mutations cause a …