Email: portico-services@ucl.ac.uk
Help Desk: http://www.ucl.ac.uk/ras/portico/helpdesk
- Associate Professor
- Institute of Ophthalmology
- Faculty of Brain Sciences


Molecular chaperone
networks are crucial for the maintenance of cellular homeostasis. The molecular
chaperones and their associated co-chaperones mediate vital roles in the cell
including the functional maturation and assembly of client proteins; the folding
of newly synthesized polypeptides; translocation through membranes; the
prevention of aggregation; and the promotion of degradation of misfolded or
non-native substrates by the ubiquitin proteasome system (UPS). My
research programme, in the Molecular and Cellular Neuroscience laboratory, is focused on the imbalance of protein folding and degradation as an underlying
mechanism of retinal degeneration and neurodegeneration. Our studies have characterized a unique proteostasis network in the specialized neuronal
photoreceptors of the retina that integrates molecular chaperone mediated
protein folding and proteasome mediated protein degradation to regulate
photoreceptor-specific protein homeostasis. Dysfunction of this system causes
the earliest and most severe form of retinal degeneration. Our studies have
also revealed the functional importance of novel components of the cellular
protein degradation systems not only in retinal degeneration, but also
neurodegenerative disorders such as Alzheimer’s disease. We are interested in
the functional characterization of these protein folding and degradation mechanisms
and their overlap in other neurodegenerative diseases, including polyglutamine
expansion diseases (Huntington’s disease and spinocerebellar ataxia type 3) and
Parkinson’s disease.