MOLECULAR IMMUNOPATHOLOGY UNIT

Unit Contact
Professor Nicholas Hunt
Discipline of Pathology
Medical Foundation Building (K25)
92-94 Parramatta Road
Camperdown, NSW, 2042

Phone:  +61 2 9036 3242(Office)
Fax: 
+61 2 9036 3286
Email:  nhunt@med.usyd.edu.au

Unit Personnel

Head of Unit
Professor Nicholas Hunt

Research Staff
Ms Tegan Arnott
Dr Chris Austin

Dr Helen Ball
Dr Andrew Mitchell
Ms Karen Say

Postgraduate Students
Ms Tareen Ho
Mr Tim Khaw
Mr James McQuillan

Research Overview
The chief areas of research currently in the Unit are the immunopathology of infectious disease (especially malaria), and the regulation of inflammatory responses.  We are interested in expression of host genes, in particular cytokines, chemokines and haem enzymes.  Some of our work is done with experimental models in mice, some with cultured cell systems and some with material obtained post-mortem from victims of malaria.

Overview
Malaria is one of the 'big three' diseases, along with HIV and tuberculosis.  More than half of the world's population lives in areas where malaria is endemic.  There are several hundred million cases each year, resulting in about 2 million deaths.  Most of the deaths occur in children aged 2 to 6 years living in sub-Saharan Africa.  Other groups at risk include pregnant women, adults in South-East Asia and visitors to malaria-endemic areas.

Life-threatening complications of malaria infection include cerebral malaria, anaemia and lung complications.  The mechanisms through which the malaria parasite causes illness in the host is a focus of research in this laboratory.

The malaria parasite spends part of its life cycle in a mosquito and part in another host.  Different species of malaria parasite can infect humans, rodents or birds.  The stage of infection during which malaria causes illness in the host is the one where it is living inside red blood cells circulating in the body.  Many malaria researchers, including us, believe that it is the response of the immune system to the presence of the parasite that leads to many of the disease complications associated with infection.  This is called "immunopathology."

Immunity against malaria parasites in the erythrocytic stage is complex.  It certainly involves the white blood cells, or leukocytes, namely neutrophils, lymphocytes and monocytes/macrophages.  These cells attempt to destroy malaria parasite through effector molecules such as perforin/granzyme, antibody and, possibly, nitric oxide.  The immune and inflammatory responses are controlled and coordinated by families of molecules called cytokines and chemokines.  Cytokines are proteins produced by cells involved in immune or inflammatory responses that act on other such cells to tell them to start carrying out a particular function, or to cease doing so.  Chemokines are cytokines that are involved in attracting leukocytes to sites of infection and inactivating them.

Although the laboratory has some interest in the role of these factors in host immunity against malaria, its chief focus is in the role that they may play in the disease complications.

Specific Projects
Host Response to Malaria
      For many years the destruction of malaria parasites during the host immune response was considered to be chiefly dependent on the production of antibody and the phagocytosis of parasitised erythrocytes, principally by macrophages in the spleen.  However, more recently it has become apparent that this scheme cannot explain all clinical observations about malaria, particularly the presence within intact erythrocytes of degenerating parasites and the extensive tissue damage seen in some host species even when the parasite burden is very low.
      Does cellular immunity contribute to the host response against malaria parasites?  It is believed that the cytokine interferon-gamma plays a key role.  This cytokine can be produced by Thelper (CD4+) lymphocytes, CD8+ lymphocytes, natural killer (NK) cells or natural killer T (NKT) cells.  Interferon-gamma may activate macrophages to produce a range of soluble mediators, some of which may be deleterious to the parasite either directly or indirectly.  Two such effector molecules that have been suggested to be directly destructive of malaria parasites are reactive oxygen species (ROS) and reactive nitrogen intermediates (RNI).
      Using a unique type of gene knockout mouse, the gp91phox-/- mouse obtained from Dr Mary Dinauer (Indianapolis), we have evaluated the role of phagocyte-derived ROS in malaria immunity in mouse models.  These gene knockout mice have been genetically engineered to inactivate the key enzyme, NADPH oxidase, that generates the first in a chain of ROS in phagocytes.  Thus these cells cannot generate anti-microbial ROS.  We have found, in work submitted for publication, that the rate of muliplication of 5 different strains of malaria parasites in these mice is unchanged from normal ("wild type") mice that possess normal phagocytes.  Thus ROS are not critical for the host immune response against primary or secondary malaria infections in mice.
      Other researchers have questioned whether there is a major role for nitric oxide in host anti-malarial responses.
Collaborators:  Dr Mary Dinaeur, Indianapolis, USA
Funding:
NHMRC
Pertinent Publications:
Potter, S.M., Sanni, L., Simasathiansophon, S., Dinauer, M.C. & Hunt, N.H. (2001). Phagocyte-derived reactive oxygen species and the immunology and pathology of murine malaria. Redox Report 6, 200-203.
Potter, S.M., Mitchell, A.J., Cowden, W., Sanni, L.A., Dinauer, M., de Haan, J. & Hunt, N.H. Do phagocyte-derived reactive oxygen species influence the progress of blood-stage malaria infections in mice? Under revision.

Roles of Cytokines and ROS in Cerebral Malaria
      Several aspects of the pathology of malaria infection seem inexplicable in terms of immunopathological reactions.  In other words, an over-vigorous, excessively prolonged or not specifically targeted host immune response may cause "bystander" damage to host cells and tissues.  The endothelium (the cells lining the blood vessels) may be particularly susceptible in this regard.  Immune system regulatory and effector molecules such as ROS, nitric oxide and cytokines have been implicated in the mechanisms leading to the pathological complications of malaria infection.
      For some years we have been studying the pathogenesis of cerebral malaria using a mouse model.  Previous indirect evidence had suggested that ROS might be important in the pathogenesis of murine cerebral malaria.  However, experiments with the gp91phox-/- mice show that phagocyte-derived ROS are not important in this process.  Like other researchers, we have shown that the cytokines interferon-gamma and lymphotoxin-alpha are important in the pathogenesis of cerebral malaria in the mouse, but another cytokine, tumour necrosis factor is not.
Collaborators:  Dr Christian Engwerda, Brisbane; Dr Jonathon Sedgwick, DNAX, USA; Professor Georges Grau, Marseille, France
Funding: NHMRC
Pertinent Publications:
Hansen, A.M., Chaudhri, G. & Hunt, N.H. (1999). Role of immune mediators in the pathology of experimental murine cerebral malaria. Redox Report 4, 321-322.
Sanni, L.A., Fu, S., Dean, R.T., Bloomfield, G., Stocker, R., Chaudhri, G., Dinauer, M.C. & Hunt, N.H. (1999). Are reactive oxygen species involved in the pathogenesis of murine cerebral malaria? J. Infect. Dis. 179, 217-222.

Biochemistry of Cerebral Malaria
     We have found that the kynurenine pathway of tryptophan metabolism is activated in the brain during cerebral and non-cerebral malaria in mice.  In this cerebral malaria model, an imbalance occurs in the production of the neuroexcitotoxin quinolinic acid relative to that of its antagonist kynurenic acid.  This may underlie the hyperexcitable phase of cerebral malaria in mice.  Following on from this, we found that there was a disturbance in the profile of the biologically active metabolites of this pathway in the cerebrospinal fluid of cerebral malaria patients from Vietnam and Malawi.
      These studies are being carried further using a unique gene knockout mouse, the indoleamine 2,3-dioxygenase gene knockout mouse from Drs Mellor and Munn in the USA.
      Our studies using Nuclear Magnetic Resonance have shown that there is a change in the metabolic profiles of brain in the later stages of murine cerebral malaria, but not in the brains of mice with severe malaria that does not involve the brain complications.  There is a build up of lactate and alanine, which classically are considered to be markers of a shift to a hypoxic pattern of metabolism.  These changes are similar to previous observations in human cerebral malaria.  However, the mechanisms involved are not clear in either the mouse model or in the human disease and we are continuing our investigations of these.  The mouse model allows us to test hypotheses about the mechanisms underlying these metabolic changes.
Collaborators:  Dr Caroline Rae, Department of Biochemistry, University of Sydney; Dr Isabelle Medana, Oxford, UK; Dr Terrie Taylor, Blantyre, Malawi; Dr Roland Stocker, University of New South Wales, Sydney
Funding: NHMRC, ARC
Pertinent Publications:
Sanni, L.A., Tattam, B., Thomas, S.R., Moore, D., Chaudhri, G., Stocker, R. & Hunt, N.H. (1998). Dramatic changes in oxidative tryptophan metabolism along the kynurenine pathway in cerebral and non-cerebral malaria. Am. J. Pathol. 152, 611-619.
Rae, C., Maitland, A., Bubb, W.A. & Hunt, N.H. (2000). Dichloroacetate (DCA) reduces brain lactate but increases brain glutamate in experimental cerebral malaria: a 13C NMR study. Redox Report 5, 141-143.
Hansen, A.M., Driussi, C., Turner, V., Takikawa, O. & Hunt, N.H. (2000). Tissue distribution of indoleamine 2,3-dioxygenase in normal and malaria-infected tissue. Redox Report 5, 112-115.
Medana, I.M., Hien, T.T., Day, N.P., Phu, N.H., Mai, N.T.H., Chu’ong, L.V., Chau, T.T.H., Taylor, A., Salahifar, H., Stocker, R., Smythe, G., Turner, G.D.H., Farrar, J., White, N.J. & Hunt, N.H. (2002). The clinical significance of cerebrospinal fluid levels of kynurenine pathway metabolites and lactate in severe malaria. J. Infect. Dis. 185, 650-656
Medana, I.M., Day, N., Salahifar, H., Stocker, R., Smythe, G., Bwanaisa, L., Njobvu, A., Kayira, K., Turner, G.D.H., Taylor, T.E. & Hunt, N.H. (2003). Metabolites of the kynurenine pathway of tryptophan metabolism in the cerebrospinal fluid of Malawian children with malaria. J. Infect. Dis. 188, 844-849
Rae, C., McQuillan, J.A., Parekh, S.P., Bubb, W.A., Weiser, S., Balcar, V.J., Hansen, A., Ball, H. & Hunt, N.H. (2004). Brain gene expression, metabolism and bioenergetics: Interrelationships in murine models of cerebral and non-cerebral malaria. FASEB J. 18, 499-510
Hansen, A.M., Ball, H.J., Mitchell, A., Miu, J.M., Takikawa, O. & Hunt, N.H. (2004). Increased expression of indoleamine 2,3-dioxygenase in murine malaria infection is predominantly localized to the vascular endothelium. Int J Parasitol 34, 1309-1319

Other Immunopathological Mechanisms in Cerebral Malaria
     It has been shown by others that CD8+ T lymphocytes play an essential role in the pathogenesis of cerebral malaria.  We have been further investigating this, concentrating on the role of effector molecules used by these cells to kill cellular targets.
     The expression of chemokines and chemokine receptors at the mRNA level in the brain has been thoroughly documented and intervention studies using appropriate gene knockout mice are under way to evaluate whether these changes are essential for the pathogenesis of cerebral malaria in this mouse model.
     Also of interest to the laboratory are the roles of the haem enzymes haem oxygenase-1 and cyclooxgenase-2.  We have shown that a selective inhibitor of cyclooxgenase-2 exacerbates the onset of cerebral malaria in the mouse model, which could be relevant to the known deleterious effects of ingesting aspirin in humans infected with malaria.
     We have started on a program of evaluating the interaction of cytokines and malaria antigens with the individual cell components that make up the brain: neurons, astrocytes, microglia and endothelial cells.  This work will largely be carried out in cell culture using mouse cells.
     A unique approach to studying cell-cell interactions and pathological mechanisms employed in our laboratory has been the use of retinal wholemounts.  Insights have been gained into the roles of adhesion molecules, astrocytes and microglia in murine cerebral malaria in collaboration with Dr Tailoi Chan-Ling, University of Sydney.
Collaborators:  Dr Tailoi Chan-Ling, Department of Anatomy and Histology, University of Sydney; Professor Iain Campbell, Department of Biochemistry, University of Sydney
Funding: NHMRC, University of Sydney Sesqui Scheme
Pertinent Publications:
Chan-Ling, T., Neill, A.L. & Hunt, N.H. (1992). Early microvascular changes in murine cerebral malaria detected using retinal wholemounts. Am. J. Pathol. 140, 1121-1130
Neill, A.L., Chan-Ling, T. & Hunt, N.H. (1993). Comparisons between microvascular changes in cerebral malaria in mice, using the retinal wholemount technique. Parasitology 107, 477-487
Medana, I.M., Chan-Ling, T. & Hunt, N.H. (1996). Redistribution and degeneration of retinal astrocytes in experimental murine cerebral malaria: relationship to disruption of the blood-retinal barrier. Glia 16, 51-64. [Front cover photograph].
Ma, N., Hunt, N.H., Madigan, M.C. & Chan-Ling, T. (1996). Correlation between enhanced vascular permeability, upregulation of cell adhesion molecules and monocyte adhesion in retina during fatal murine cerebral malaria. Am. J. Pathol. 149, 1745-1762.
Medana, I.M., Hunt, N.H. & Chan-Ling, T. (1997). Early activation of microglia in the pathogenesis of fatal murine cerebral malaria. Glia 19, 91-103. [Front cover photograph].
Ma, N., Madigan, M.C., Chan-Ling, T. & Hunt, N.H. (1997). Compromised blood-nerve barrier, astrogliosis, and myelin disruption in optic nerves during fatal murine cerebral malaria. Glia 19, 135-151.
Medana, I.M., Chan-Ling, T. & Hunt, N.H. (2000). Reactive changes of retinal microglia during fatal murine cerebral malaria: effects of dexamethasone and experimental permeabilisation of the blood-brain barrier. Am. J. Pathol. 156, 1055-1065.
Medana, I.M., Chaudhri, G., Chan-Ling, T. & Hunt, N.H. (2001). Central nervous system in cerebral malaria: "innocent bystander" or active participant in the induction of pathology? (Review). Immunol. Cell Biol. 79, 101- 120.
Ball, H.J., MacDougall, H.G., McGregor, I.S. & Hunt, N.H. (2004). Cyclooxygenase 2 in the pathogenesis of cerebral malaria. J. Infect. Dis. 189, 751-758.

Gene Expression in Cerebral Malaria
     As noted earlier, the host immune response has been found to contribute to the pathogenesis of cerebral malaria.  Determining which components of the immune response are induced might lead to additional therapies.  We are investigating changes in gene expression in specific cell types in murine malaria infection, and carrying out correlative studies where possible on human brain tissue obtained post-mortem from malaria victims.  A feature of cerebral malaria is damage to the endothelial cells leading to the breakdown of the blood-brain barrier.  We have isolated endothelial cells from mouse brain using an advanced technique called laser capture microdissection.  This allows individual cell types to be captured from frozen sections of tissue then extracted for analysis of mRNA and, thereby, gene expression.  We hope to extend this technique to human post-mortem tissue in collaboration with Dr Terrie Taylor, Malawi.
Collaborators:  Dr Terrie Taylor, Blantyre, Malawi
Funding: NHMRC, University of Sydney Sesqui Scheme
Pertinent Publications:
Ball, H.J., McParland, B., Driussi, C. & Hunt, N.H. (2002). Isolating vessels from the mouse brain for gene expression analysis using laser capture microdissection. Brain Research Protocols 9, 206-213.
Rae, C., McQuillan, J.A., Parekh, S.P., Bubb, W.A., Weiser, S., Balcar, V.J., Hansen, A., Ball, H. & Hunt, N.H. (2004). Brain gene expression, metabolism and bioenergetics: Interrelationships in murine models of cerebral and non-cerebral malaria. FASEB J. 18, 499-510.

Gene Expression in Asthma
     In collaboration with Professor Judy Black, Drs Jeanette Burgess and Peter Johnson from Pharmacology, University of Sydney and Dr Margaret Hughes, Pharmacy, University of Sydney, we have examined the expression of genes in airways smooth muscle in patients with asthma.  This led to the identification of a role for the cell surface molecule OX40L.  The therapeutic possibilities of this have been explored in collaboration with a major pharmaceutical company.
Collaborators:  Professor Judy Black, Dr Janette Burgess, Dr Peter Johnson, Department of Pharmacology, University of Sydney; Dr Margaret Hughes, Pharmacy, University of Sydney
Funding: Australian Research Council SPIRT, NHMRC (Prof J Black)
Pertinent Publications:
Burgess, J.K., Carlin, S., Pack, R.A., Arndt, G.M., Au, W. W., Johnson, P.R.A., Black, J.L. & Hunt., N.H. (2004). Detection and characterisation of OX40 ligand expression in human airway smooth muscle cells: A possible role in asthma? J. Allergy Clin. Immunol. 113, 683-689.
Burgess, J.K., Blake, A.E., Boustany, S., Johnson, P.R.A., Armour, C.L., Black, J.L., Hunt, N.H. & Hughes, J.M. (2005). Increased expression of CD40 and OX40L on asthmatic airway smooth muscle following stimulation with TNFa: Modulation by IL1b. J. Allergy Clin. Immunol. 115, 302-308.

Inflammation
     We are collaborating with Dr Bob Bao of this Department in his work on the pathogenesis of inflammatory bowel disease using mouse models.
Collaborators:  A/Prof Bob Bao, Mucosal Immunology Unit, Department of Pathology, University of Sydney

Honours Students Projects Available in 2009
Understanding a key new enzyme in the kynurenine pathway
Supervisors:  Dr Helen Ball (Pathology), Professor Nick Hunt (Pathology)
Contact Details: Level 1, Medical Foundation Building helenb@med.usyd.edu.au; phone: 9036 3238

Project Description:  Indoleamine 2,3-dioxygenase (IDO) catalyses the first step in the kynurenine pathway of tryptophan metabolism. This pathway is involved in numerous physiological and pathophysiological processes including immunomodulation and central nervous system disorders. We recently discovered a second enzyme (IDO2) that performs the same reaction as IDO (now IDO1) but which is found in different anatomical locations. This project will further investigate the expression pattern of IDO2 by looking at subcellular localisation and expression during development and disease states. The biochemical properties of IDO2 will be examined by determining whether IDO2 enzymatic activity in intact cells is inhibited by nitric oxide, an important mechanism for regulation of IDO1 activity. This project will possibly also include investigating phenotypical characteristics of an IDO2 gene knockout mouse e.g. susceptibility to infectious disease (malaria and meningitis). Laboratory techniques involved in this project include immunohistochemistry, immunofluorescence, quantitative RT-PCR, cell culture, transfection, biochemical assays, mouse models of disease.

What causes death and disability in bacterial meningitis?
Supervisors:  Professor Nick Hunt and Dr Helen Ball (Pathology)
Contact Details: Level 1, Medical Foundation Building (K25); email: nhunt@pathology.usyd.edu.au; helenb@med.usyd.edu.au 

Project Description:   Bacterial Meningitis kills over 150,000 people every year and many more suffer neurological sequelae following infection.  We have exetensive experience in uncovering the immunopathological mechanisms in cerebral malaria. We have recently established a mouse model of bacterial meningitis and our preliminary evidence suggests that the cytokine interferon gamma (IFNg) plays an important role in determining whether mice with bacterial meningitis succumb to the disease. IFNg is a key regulator of indoleamine 2,3-dioxygenase-1 (IDO1) expression and thus activity of the kynurenine pathway. Activation of this pathway is observed in a number of central nervous system disorders including cerebral malaria.
This project will further investigate the role of IFNg in bacterial meningitis. Gene expression during bacterial meningitis in IFNg gene knockout and wildtype mice will be compared using gene arrays to identify pathways regulated by IFNg in bacterial meningitis. IDO1 expression is upregulated in bacterial meningitis in wildtype mice and IDO1 expression will also be investigated in the IFNg gene knockout mice. The levels of kynurenine pathway metabolites during bacterial meningitis will be measured in both mouse strains. The neurological sequelae in IFNg gene knockout mice that recover from bacterial meningitis will be measured in behavioural studies that compare these mice with both uninfected and antiobiotic-treated mice. The progression and outcome of bacterial meningitis in IDO1 gene knockout mice will also be investigated. Laboratory techniques involved in the project include mouse models of disease, mouse behavioural studies, quantitative RT-PCR and gene arrays, histopathological analysis, biochemical assays.

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Recent Publications

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Current Student Research Projects
Mr James McQuillan
Severe malaria is an infection tht poses a two pronged problem for the patient.  The parasite continues to replicate, invading host red blood cells and destroying them.  At the same time the immune system, in an attempt to eradicate the parasite can often have deleterious side-effects.  We plan to investigate the outcome of combining immune modulation strategies with anti-malarial drugs in an attempt to improve treatment for malaria.  We will also study the biological mechanisms underlying these actions.

Unit Resources

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Grants Awarded

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