Medical Director, Neurology Clinical Research UnitDepartments / Divisions
Durham, NC 27710
Dementia, Alzheimer's disease, memory disorders, inherited neurodegenerative diseases
My research focuses on the molecular mechanisms that are responsible for pathogenesis in the CAG trinucleotide repeat diseases. Eight inherited neurodegenerative diseases are known to be caused by expansion of the CAG repeat and all are characterized by expression of an expanded polyglutamine domain in the disease protein. The major interest of my laboratory is characterizing cellular processes leading to pathogenesis of the polyglutamine repeat diseases and developing new therapies.
Current work focuses on the role of mitochondria in pathogenesis and the role played by cellular stress responses. We have also identified a peptide that blocks pathogenesis in cell culture and fly models of disease and will be studying the effect of these peptides in mice.
We are also working on developing therapies for Parkinson's disease by identifying peptide inhibitors of alpha synuclein aggregation.
My area of expertise is neurodegenerative diseases and dementia with an emphasis on the CAG trinucleotide repeat diseases (Huntington's disease, dentatorubral-pallidoluysian atrophy (DRPLA), Haw River Syndrome; spinocerebellar ataxia type 1, 2, 3 (Machado-Joseph disease), 6 and 7) and Alzheimer's disease.
Keywords: Trinucleotide repeats; polyglutamine; neurodegenerative disease; dementia; protein-protein interactions; Alzheimer's disease; Parkinson's disease; Huntington's disease.
This faculty member (or a member of their immediate family) has a working relationship (i.e. consulting, research, and/or educational services) with the companies listed below. These relations have been reported to the health system leadership and, when appropriate, management plans are in place to address potential conflicts.
Heinzen, EL; Need, AC; Hayden, KM; Chiba-Falek, O; Roses, AD; Strittmatter, WJ; Burke, JR; Hulette, CM; Welsh-Bohmer, KA; Goldstein, DB. Genome-wide scan of copy number variation in late-onset Alzheimer's disease. Journal of Alzheimer's Disease. 2010;19:69-77. (2010) Abstract
Wang, CS; Burke, JR; Steffens, DC; Hulette, CM; Breitner, JC; Plassman, BL. Twin pairs discordant for neuropathologically confirmed Lewy body dementia. Journal of Neurology, Neurosurgery and Psychiatry. 2009;80:562-565. (2009) Abstract
Gambetti, P; Dong, Z; Yuan, J; Xiao, X; Zheng, M; Alshekhlee, A; Castellani, R; Cohen, M; Barria, MA; Gonzalez-Romero, D; Belay, ED; Schonberger, LB; Marder, K; Harris, C; Burke, JR; Montine, T; Wisniewski, T; Dickson, DW; Soto, C; Hulette, CM; Mastrianni, JA; Kong, Q; Zou, WQ. A novel human disease with abnormal prion protein sensitive to protease. Annals of Neurology. 2008;63:697-708. (2008) Abstract
Plassman, BL; Langa, KM; Fisher, GG; Heeringa, SG; Weir, DR; Ofstedal, MB; Burke, JR; Hurd, MD; Potter, GG; Rodgers, WL; Steffens, DC; McArdle, JJ; Willis, RJ; Wallace, RB. Prevalence of cognitive impairment without dementia in the United States. Annals of Internal Medicine. 2008;148:427-434. (2008) Abstract
Plassman, BL; Langa, KM; Fisher, GG; Heeringa, SG; Weir, DR; Ofstedal, MB; Burke, JR; Hurd, MD; Potter, GG; Rodgers, WL; Steffens, DC; Willis, RJ; Wallace, RB. Prevalence of dementia in the United States: the aging, demographics, and memory study. Neuroepidemiology. 2007;29:125-132. (2007) Abstract
Langa, KM; Plassman, BL; Wallace, RB; Herzog, AR; Heeringa, SG; Ofstedal, MB; Burke, JR; Fisher, GG; Fultz, NH; Hurd, MD; Potter, GG; Rodgers, WL; Steffens, DC; Weir, DR; Willis, RJ. The Aging, Demographics, and Memory Study: study design and methods. Neuroepidemiology. 2005;25:181-191. (2005) Abstract
Burke, JR; Enghild, JJ; Martin, ME; Jou, YS; Myers, RM; Roses, AD; Vance, JM; Strittmatter, WJ. Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH. Nature Medicine. 1996;2:347-350. (1996) Abstract
Burke, JR; Wingfield, MS; Lewis, KE; Roses, AD; Lee, JE; Hulette, C; Pericak-Vance, MA; Vance, JM. The Haw River syndrome: dentatorubropallidoluysian atrophy (DRPLA) in an African-American family. Nature Genetics. 1994;7:521-524. (1994) Abstract