James R. Burke, MD, PhD
- Medicine / Medicine-Neurology
- Psychiatry
Address
DUMC 2900
Durham, NC 27710
Appointment Telephone
919-668-7600
Office Telephone
919-668-5273
Fax Telephone
919-684-6514
- MD, State University of New York, 1985
- Neurology, Duke University Medical Center, 1986-1989
- Aging, Duke University Medical Center, 1989-1990
- PhD, Biochemistry, Oak Ridge National Lab, University of Tennessee, 1981
Clinical Interests
Dementia, Alzheimer's disease, memory disorders, inherited neurodegenerative
diseases
Research Interests
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 physician (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.
- Accera
- Bristol-Myers Squibb
- Decision Resources, Inc.
- Gerson Lehrman Group, Inc.
- GlaxoSmithKline
- High Point Pharmaceuticals
- Medscape ( WebMD HLTH Corp.)
- TCL Institute
- TransTech Pharma, Inc.
Representative Publications
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. J Alzheimers Dis. 2010 Jan;19(1):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. J Neurol Neurosurg Psychiatry. 2009 May;80(5):562-5.
(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. Ann Neurol. 2008 Jun;63(6):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. Ann Intern Med. 2008 Mar 18;148(6):427-34.
(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(1-2):125-32.
(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(4):181-91.
(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. Nat Med. 1996 Mar;2(3):347-50.
(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. Nat Genet. 1994 Aug;7(4):521-4.
(1994)
Abstract
