Core B: Bioenergetics Mitochondrial dysfunction has long been a consistent observation in Parkinson's disease (PD). To understand the consequences of PD disease causing genetic mutations on the function of mitochondria the Bioenergetics Core B will provide the following analyses to the three projects in this program including: Measuring rates of respiration, oxygen consumption and ATP generation, Measuring calcium dynamics, Measuring reactive oxygen and reactive nitrogen species, measuring the activity of the electron transport chain enzymes and metabolic enzymes, and Measuring plasma versus mitochondria! membrane potential and mitochondria! membrane permeability. The Bioenergetics Core B is a shared resource of the Program. It will measure mitochondria! function, membrane permeability and membrane potential in the presence of AIMP2 and characterize mitochondria! enzymes, oxygen consumption and ATP concentrations in the conditional AIMP2 transgenic mice for Project 1. For Project 2 the Core will measure electron transport chain enzymes and metabolic enzymes and reactive oxygen and reactive nitrogen species in the various models of alpha-synuclein mediated neuronal injury. For Project 3 all measures of mitochondria! function will be conducted to characterize the LRRK2 knockout and LRRK2 transgenic mice. Thus this Core will play a central and integrated role in the investigations in the pathogenesis of PD of this Center.

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Specialized Center (P50)
Project #
5P50NS038377-15
Application #
8533022
Study Section
National Institute of Neurological Disorders and Stroke Initial Review Group (NSD)
Project Start
Project End
Budget Start
2013-08-01
Budget End
2014-07-31
Support Year
15
Fiscal Year
2013
Total Cost
$96,153
Indirect Cost
$37,523
Name
Johns Hopkins University
Department
Type
DUNS #
001910777
City
Baltimore
State
MD
Country
United States
Zip Code
21218
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Nucifora Jr, Frederick C; Nucifora, Leslie G; Ng, Chee-Hoe et al. (2016) Ubiqutination via K27 and K29 chains signals aggregation and neuronal protection of LRRK2 by WSB1. Nat Commun 7:11792
Mills, Kelly A; Mari, Zoltan; Pontone, Gregory M et al. (2016) Cognitive impairment in Parkinson's disease: Association between patient-reported and clinically measured outcomes. Parkinsonism Relat Disord 33:107-114

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