Biochemistry; Biophysics; Cell Biology; Dystonia Musculorum Deformans
Molecular Virology: Virology laboratories
Mechanisms of membrane dynamics in relation to nuclear envelopathies and viral infection
Nuclear envelopathies are a diverse group of congenital diseases that are caused by mutations affecting proteins in the nuclear envelope or lamina. We hypothesize that envelopathy-associated alleles act at least in part through a gain of function mechanism that leads to a poisoning of nuclear membrane dynamics. Our goal is to unravel the cellular mechanisms that regulate protein homeostasis in the nuclear periphery, and to elucidate the role that these pathways play in muscular dystrophies, premature aging and related envelopathies. We exploit viral proteins known to manipulate the nuclear envelope as a novel approach to identify cellular factors involved in protein turnover and non-canonical nuclear transport via vesicular intermediates.
Specialized Terms: DYT1 Dystonia, Membrane dynamics; Torsin ATPases; Alternative nuclear transport (nuclear egress); Protein quality control; viral pathogenesis
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- Chenguang Zhao, Rebecca S. H. Brown, Anna R. Chase, Markus R. Eisele, and Christian Schlieker. Regulation of Torsin ATPases by LAP1 and LULL1 PNAS 2013. doi:10.1073/pnas.1300676110
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- Rose AE, Brown R and C. Schlieker . Torsin ATPases - not your typical AAA+ ATPase. Critical Reviews in Biochemistry and Molecular Biology (2015), in press. http://www.tandfonline.com/doi/full/10.3109/10409238.2015.1091804#.Vh-wDaQViKA