Associate Sr. Fellow,
- Professor, Dept. of Biochemistry, Molecular Biology & Cell Biology, Northwestern University
Department of Biochemistry, Molecular Biology & Cell Biology
2205 Tech Drive, Hogan 2-100
Evanston, IL 60208
Phone: 847 491 3714
Fax: 847 491 3714
Understanding the principles underlying CELLULAR QUALITY CONTROL — the integration of processes by which the cell senses, responds and adapts to environmental and physiological challenges — is among the most fascinating problems in biology. The appearance of incorrectly expressed or improperly folded proteins results in a cellular stress response involving activation of stress-induced transcription factors and leads to the elevated expression of molecular chaperones and proteases that serve to clear damaged proteins.
An imbalance in protein homeostasis results in the accumulation of misfolded and aggregation-prone proteins that are poorly refolded and degraded, often accumulating as oligomeric intermediate species and aggregates in different subcellular compartments. These events are hallmarks of human genetic diseases including the polyglutamine-expansion diseases such as Huntington’s disease, Parkinson’s disease, Alzheimer’s disease, familial ALS, prion diseases, amyloidosis, cystic fibrosis, and a-1-antitrypsin disease. This has led to increased interest in the toxicity and pathogenesis of misfolded proteins and the role of protein aggregates in cellular dysgenesis.
Our laboratory is interested in the fundamental events that underlie the appearance of misfolded proteins and their consequence to protein homeostasis, cellular function, and organismal adaptation and survival.