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Kinuko I. Suzuki, MD
Professor
 
Office: 919-966-4585
E-mail: kinuko_suzuki@med.unc.edu
 
 
 
 
Research Interests
 
The investigation in our laboratory is focused on the study of the pathobiology of genetic neurodegenerative disorders affecting infants and children and to explore possible therapeutic means of such disorders. Using naturally occurring murine models or models which were generated by targeted gene disruption (transgenic or knockout mice), our laboratory has been investigating the natural disease processes, with particular focus on neuronal as well as glial cell reactions associated with the progression of the pathology in the central as well as in the peripheral nervous system. The murine models currently under investigation are models of Krabbe disease, Niemann-Pick disease type C, and Gangliosidoses.

In collaboration with other laboratories, we also plan to explore the feasibility of gene transfer as a treatment of genetic diseases using these murine mutants as models. The studies include in vitro, in vivo and ex vivo approaches. The result of these studies will be carefully monitored with morphology, immunocytochemistry, biochemistry and molecular biological studies. For the treatment of genetic diseases, transfer of the missing gene into appropriate tissues/cells is an obvious treatment of choice. However, in practice, there are many problems in transferring genes, in particular for the treatment of genetic diseases affecting the nervous system. Therefore, well controlled studies using established animal models of the human disease is essential before any attempt of gene therapy in human patients.
 
Selected Publications
 

Mohri I, Taniike M, Taniguchi H, Kadoyama K, Kanekiyo T, Aritake K, Kushi A, Sasai H, Inui T, Eguchi N, Kanaoka Y, Ozono K, Narumiya S, Suzuki K, Urade Y. Suppression of astrogliosis by genetic depletion of hematopoietic prostaglandin D synthase or DP receptor in the demyelination mutant twitcher. J Neuroscience, (In press).

Mohri I, Taniike M, Okazaki I, Kagitani-Shimono K, Aritake K. Kanekiyo T, Yagi T, Takikita S, Kim, HyS, Urade Y, Suzuki K. Lipocalin-type prostaglandin D synthase is up-regulated in oligodendrocytes in lysosomal storage diseases and binds gangliosides. J Neurochemistry, (In press).

Suzuki K. Suzuki K. Lysosomal diseases. In Greenfield's Neuropathology, Love S, Louis D, Ellison D (eds). 8th edition ,Arnold pub Ltd, London, UK, Chapter 8, (In press).

Kagitani-Shimono K, Mohri I, Oda H, Ozono K, Suzuki K, Urade Y, Taniike M. Lipocalin-type prostaglandin D synthase (?-trace) is upregulated in the ?B-crystallin-positive oligodendrocytes and astrocytes in the chronic multiple sclerosis. Neuropath & Appl Neurol 32:64-73, 2006.

Kagitani-Shimono K, Mohri I, Fujitani Y, Suzuki K, Ohzono K, Taniike M, Urade Y. Anti-inflammatory therapy by ibudilast, a phosphodiesterase inhibitor, in demyelination of twitcher, a genetic demyelination model. Journal of Neuroinflammation 2:10-, 2005.

Yagi T, Matsuda J, Tomonaga K, Suzuki K, Suzuki K. Hematopoietic cell transplantation ameliorates clinical phenotype and progression of the CNS pathology in the mouse model of late onset Krabbe disease. J Neuropathol Exper Neurol 64:565-575, 2005.

Wu Y-P, Mizukami H, Matsuda J, Saito Y, Proia R.L. Suzuki K. Apoptosis accompanied by up-regulation of TNF-a death pathway genes in the brain of Niemann-Pick type C disease. Mol Genet Metab 84:9-17, 2005.

Walkley SU, Suzuki K. Consequences of NPC1 and NPC2 loss of function in mammalian neurons. Biochim Biophys Acta 1685:48-62, 2004.

Yagi T., Matsuda J, Takikita S., Mohri I., Suzuki K., Suzuki K. Comparative clinico-pathological study of saposin A deficient (SAP-A-/-) and Twitcher mice. J Neuropathol Exper Neurol 63:721-734, 2004

Takikita S, Fukuda T., Mohri I., Yagi T., Suzuki K. Perturbed myelination process of premyelinating oligodendrocytes in Niemann-Pick Type C mouse. J Neuropathol Exper Neurol 63:660-673, 2004.

Mason JL, Towes A, Hostettler J.D., Morell P, Suzuki K, Matsushima G.K. Oligodendrocytes and progenitors become progressively depleted within chronically demyelinated lesions. Am J Pathol 164: 1674-82, 2004.

Yagi T, McMahon E J, Takikita S, Mohri I, Matsushima G. K. Suzuki K. Fate of donor hematopoietic cells in demyelinating mutant mouse, twitcher, following transplantation of GFP+ bone marrow cells. Neurobiology of Disease 16: 98-109, 2004.

Saito Y, Suzuki K, Hulette C, Murayama S. Aberrant phosphorylation of a-synuclein in human Niemann-Pick type C1 disease. J Neuropathol Exp Neurol 63:323-328, 2004.

Vanier MT, Saito Y, Murayama S, Suzuki K. Niemann-Pick type C disease. In Developmental Neuropathology, Golden JA, Harding BN (eds), International Society of Neuropathology, Pathology & Genetics series, Chapter30, 283-286, 2004.

Suzuki K, Vanier MT. Sphingolipidosis. In Developmental Neuropathology, Golden JA, Harding BN (eds), International Society of Neuropathology, Pathology and Genetics series, Chapter 28, 235-266, 2004.

Walkley S, Suzuki K. NPC1 loss of function in mammalian neurons. In special issue on Niemann-Pick type C, Liscum L and Sturley S (Eds), Molecular and cell biology of lipids, BBActa 1685, 48-62, 2004.

Arnett HA, Wang Y, Matsushima GK, Suzuki K, Ting JP-Y. Functional genomic analysis of remyelination reveals importance of inflammation in oligodendrocyte regeneration. J Neuroscience 23:9824-9832, 2003.

Trebst C, Staugaitis S, Tucky B, Wei T, Suzuki K, Aldape KD, Pardo CA, Troncoso J, Lassmann H, Ransohoff RM. Chemokine receptors on infiltrating leukocytes in inflammatory pathologies of the central nervous system (CNS) Neuropath Appl Neurobiol 29:584-595, 2003.

Mohri I, Eguchi N, Suzuki K, Urade Y, Taniike M. Hematopoietic prostaglandin D synthase is expressed in microglia in the developing postnatal mouse brain. Glia 42: 263-274, 2003.

Suzuki K, Ezoe T, Tohyama J, Matsuda J, Vanier MT, Suzuki K. Are animal models useful to understand the pathophysiology of lysosomal disease? Proceedings of the 3rd TKT International Symposium on Lysosomal Disease. Acta Paediatrica Suppl 443:54-62, 2003.

Bu B, Klunemann H, Suzuki K, Husseman J, Bird T, Jin L-W, Nochin D, Vincent I. Why are Purkinje neurons resistant to neurofibrillary tangle formation? Clues from Niemann-Pick Type C disease Neurobiol Dis 11:285-297, 2002.

McMahon EJ, Suzuki K, Matsushima GK. Peripheral macrophage recruitment in cuprizone-induced CNS demyelination despite an intact blood-brain barrier. J Neuroimmunol 130:32-45, 2002.

Ishibashi T, Dupree JL, Ikenaka K, Hirahata Y, Honke K, Peles E, Popko B, Suzuki K, Nishino H, Baba H. A myelin galactolipid, sulfatide, is essential for maintenance of ion channels on myelinated axon but not essential for initial cluster formation. J Neurosci 22:6507-14, 2002.

Saito Y, Suzuki K, Namba E, Yamamoto T, Ohno K, Murayama S. Niemann-Pick type C disease: Accelerated neurofibrillary tangle formation and amyloid ? deposition associated with APOE ?4 homozygocity. Ann Neurol 52: 351-355, 2002.

Saito Y, Geyer A, Sasaki R, Kuzuhara S, Namba E, Miyasaka T, Suzuki K, Murayama S. Early onset rapidly progressive familial tauopathy with R406W mutation. Neurology 58: 811-813, 2002.

Honke K, Hirahara Y, Dupree J, Suzuki K, Popko B, Fukushima K, Fukushima J, Nagasawa T, Yoshida N,Wada Y, Taniguchi N. Paranodal junction formation and spermatogenesis require sulfoglycolipids. Proc Natl Acad Sci USA 99:4227-4232, 2002.

Arnett HA, Hellendall RP, Matsushima GK, Suzuki K, Laubach VE, Sherman P, Ting J P-Y. The protective role of inducible nitric oxide in a neurotoxicant-induced demyelinating model. J Immunol168: 427-433, 2002.

Taniike M, Mohri I, Eguchi N, Beuckmann CT, Suzuki K, Urade Y: Perineuronal oligodendrocytes protect against neuronal apoptosis through the production of lipocalin-type prostaglandin D synthase in a genetic demyelinating model.. J Neurosci 22:4885-96, 2002.

Suzuki K. Section 1. 3 The nervous system: pattern of tissue pathology in neurological diseases. In: Universes in delicate balance: chemokines and the nervous system. Ransohoff R, Suzuki K, Proudfoot AEI, Hickey WF, Harrison JK (eds), Elservier Publish Co, Amsterdam, the Netherlands, 49-55, 2002.

Suzuki, K. Section 1. 2 The nervous system: cellular reaction to insult (Cellular neuropathology). In: Universes in delicate balance: chemokines and the nervous system, Ransohoff, R, Suzuki K, Proudfoot AEI, Hickey WF, Harrison JK (eds). Elservier Publish Co, Amsterdam, the Netherlands, 31-48, 2002.

Suzuki, K. Suzuki, K. Lysosomal disease. In Greenfield's Neuropathology, Latos, PL, Graham, DI (eds). 7 th edition, Arnold pub Ltd, London, UK, Chapter 11, 658-735, 2002.

Arnett HA, Mason JL, Marino M, Suzuki K, Matsushima GK, Ting JP-Y. TNF a signaling through TNFR2 promotes proliferation of oligodendrocyte progenitors and remyelination. Nature Neurosci 4:1116-1122, 2001.

Matsuda J, Vanier MT, Saito Y, Suzuki K, Suzuki K. Dramatic phenotypic improvement during pregnancy in a genetic leukodystrophy: High estrogen level appears to be a critical factor. Human mol genet. 10: 2709-2715, 2001.

Wu, T-P, McMahon EJ, Matsuda J, Suzuki K, Matsushima GK, Suzuki K. Expression of immune-related molecules is down regulated in twitcher mice following bone marrow transplantation. J Neuropath Exp Neurol 60: 1062-1074, 2001.

 
 
 
 
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