My main research interests revolve around
understanding complex biological systems at the molecular and atomic level. In
my laboratory we study the structure and function of protein biomolecules in an
effort to understand their role, as well as the role of their disease-causing
variants (such as rare mutations or single nucleotide polymorphisms), in the
pathogenesis, predisposition and diagnosis of human disease. We furthermore aim
to validate and complement existing genetic or clinical association studies by
providing mechanism-based insight that can establish the diagnostic and
prognostic value of protein biomarkers for human diseases. We are also involved
in rational-drug design efforts focused on the protein molecules we are
currently investigating. We guide and support these efforts by providing
structural and mechanistic insight as well as developing and performing
customised in vitro and cell-based assays.
EXPERIMENTAL APPROACHES
We utilize a variety of biochemical approaches in an effort to unravel the molecular-level or atomic-level mechanisms that underlie protein function in health and disease. These approaches include:
Recombinant protein expression in bacterial, insect and mammalian cell lines (small and medium scale)
Protein purification and characterization (FPLC, SDS-PAGE etc)
Gene cloning and expression vector construction
Biophysical and structural analysis of proteins
Enzymology and assay development
Proteomics and peptide sequencing
Specialized cell-based assays
Current research projects:
ER AMINOPEPTIDASES AND THE IMMUNE RESPONSE
The adaptive immune response relies on the
presentation of small peptide-antigen fragments onto specialised cell-surface
receptors (MHC molecules) for recognition by cytotoxic T-lymphocytes (for more
information click here). These peptides are generated from the degradation of
intra or extra cellular proteins. Intracellular aminopeptidases like ERAP1,
ERAP2 and IRAP play a crucial role in antigenic peptide generation and affect
quantitative and qualitative aspects of the immune response (for more
information click here, also read this paper and this paper).
Naturally occurring polymorphisms (SNPs) on
ERAP1/2 have been linked with predisposition to autoimmune diseases like
Ankylosing Spondylitis and Diabetes as well as susceptibility to viral and
bacterial infections and tumour development. How ERAP1 SNPs affect disease
predisposition and pathogenesis in not known, but it has been hypothesised that
aberrant antigen presentation may be the mechanism behind these effects. We are
currently evaluating the diagnostic and prognostic value of these SNPs by
studying their effects on protein function and antigen processing. We are also
developing small molecular-weight inhibitors for these molecules in an effort to
control their enzymatic activity in vivo for immunotherapy applications.
HUMAN APOLIPOPROTEINS AND DISEASE
Apolipoprotein E (apoE) is an important
protein of the lipid transport system that has an indisputable role in
atherosclerosis, dyslipidemia, and Alzheimer's disease (AD) (for more
information on apolipoproteins click here). The apoE4 isoform is a major
genetic risk factor for AD with 40% of all patients having at least one apoE4
allele. ApoE4 is susceptible to proteolysis in the brain and apoE4 N-terminal
fragments have been found in the brain of AD patients. It is possible that the
presence of some of these fragments are of significant prognostic value for the
development of AD. ApoE4 displays significant structural plasticity and
undergoes dramatic conformational changes upon binding lipids.
In a recent study we discovered that fragments of apoE4 are
actually more stable than the wild-type protein and therefore lack the
necessary conformational plasticity for function. Our results also suggest a
complex but labile tertiary structural organization within the C-terminal
moiety of apoE4 that may be important for its physiological function.
Alterations of the structure of apoE4 brought forth by truncations may be
responsible for the abnormal apoE4 functionality observed in Alzheimer disease
patients brain (Biochemistry,
2008). A
specific fragment of apoE4 has been recently discovered to be able to promote
the internalisation of ABeta42 peptide (the principle component of amyloid
plaques that are found in the brain of AD patients) into neuronal cells. This
finding provides a possible link between two early molecular events in AD
pathogenesis, namely apoE4 proteolysis and Abeta42 presence in neuronal cells (J.
Neurochem., 2010).
More details on ongoing research projects can
be found below: