The Potamkin Prize is one of the most prestigious awards for researchers working on Pick’s, Alzheimer’s, and related diseases. This year the prize goes to Lennart Mucke and Bruce Miller, who both have labs at the University of California, San Francisco. The award, sponsored by the Potamkin family of New York, Philadelphia, and Miami, was presented at the 62nd annual meeting of the American Academy of Neurology, held this week in Toronto, Canada.
Mucke, who is no stranger to Alzforum readers, was rewarded for his work relating amyloid-β and tau, hallmarks of Alzheimer disease (AD), to neural toxicity and the disruption of neural networks. His work has linked a variety of molecular players, including cytokines (ARF related news story and Wyss-Coray et al., 1997), calcium-binding proteins (see ARF related news story), Aβ-degrading enzymes (see ARF related news story) and even collagen, typically associated with connective tissue (see ARF related news story), to AD pathology in animal models of the disease. His discovery that mice overexpressing human Aβ have spontaneous non-epileptic seizures induced by hyperactive neurons, and a compensatory elevation in inhibitory neural network activity, offered a new rationale for Aβ toxicity (see ARF related news story), and a possible explanation for epileptic seizures in AD patients.
Miller, a behavioral neurologist, is a world leader in the study and treatment of patients with frontotemporal lobar degeneration (FTLD), also called Pick disease. FTLD is a rare form of dementia characterized by deposits of tau (Pick bodies) in the frontal and temporal lobes of the brain. Miller’s work has helped uncover genetic mutations and inheritance patterns (see Goldman et al., 2005) that increase risk for the disease and characterize different forms of FTLD (see, e.g., Johnson et al., 2005 and Miller, 2007). “He is a clinician scientist who has been able to bring together collaborators to solve the complex clinical heterogeneity of this group of disorders so that one can begin to plan clinical trials and understand how to use imaging and biomarker diagnostics to separate out complex phenotypes,” said John Trojanowski, University of Pennsylvania, Philadelphia. Mucke and Miller will share the $100,000 Potamkin Prize, which will go toward further dementia research.—Tom Fagan.
- TGF-β1 Linked to Plaque Formation
- Calbindin Study: Is Calcium the Molecular Handle on Dysfunction in AD?
- Metalloproteases—A Shining Challenge to Aβ
- Sticking It to Oligomers—Does Collagen Protect Neurons From Aβ?
- Do "Silent" Seizures Cause Network Dysfunction in AD?
- Wyss-Coray T, Masliah E, Mallory M, McConlogue L, Johnson-Wood K, Lin C, Mucke L. Amyloidogenic role of cytokine TGF-beta1 in transgenic mice and in Alzheimer's disease. Nature. 1997 Oct 9;389(6651):603-6. PubMed.
- Goldman JS, Farmer JM, Wood EM, Johnson JK, Boxer A, Neuhaus J, Lomen-Hoerth C, Wilhelmsen KC, Lee VM, Grossman M, Miller BL. Comparison of family histories in FTLD subtypes and related tauopathies. Neurology. 2005 Dec 13;65(11):1817-9. PubMed.
- Johnson JK, Diehl J, Mendez MF, Neuhaus J, Shapira JS, Forman M, Chute DJ, Roberson ED, Pace-Savitsky C, Neumann M, Chow TW, Rosen HJ, Forstl H, Kurz A, Miller BL. Frontotemporal lobar degeneration: demographic characteristics of 353 patients. Arch Neurol. 2005 Jun;62(6):925-30. PubMed.
- Miller BL. Frontotemporal dementia and semantic dementia: anatomic variations on the same disease or distinctive entities?. Alzheimer Dis Assoc Disord. 2007 Oct-Dec;21(4):S19-22. PubMed.