Network of Centres of Excellence in Neurodegeneration (CoEN)

Carbon-Model: Carbon metabolism systems analysis for the identification of diseaseand patient-specific metabolic and energetic defects in neurodegenerative diseases.

Project Description:

Nerve cells rely heavily on a sufficient supply of carbon sources such as glucose and
amino acids to function, as they are highly specialised cells with limited ability to store
energy. Defects in the supply and/or proper usage of carbon sources have been
identified in several neurodegenerative disorders. These may represent primary,
disease-causing defects, or may critically contribute to disease processes that ultimately
lead to the degeneration of nerve cells. CARBON-MODEL will develop an integrated
platform to pin-point defects in carbon metabolism and energy production in nerve cells.
The approach taken is designed to be applicable to multiple types of neurodegenerative
disorders or individual patient data where new diagnostic, prognostic and stratification
tools are required. CARBON-MODEL will dissect the individual molecular components that regulate carbon metabolism using preclinical disease models and induced pluripotent
stem cell (iPSC)-derived neurons, and will implement computational approaches to fully
integrate and systematically analyse these data. The CARBON-MODEL systems platform will enable the development of novel biomarkers for diagnosis and the implementation of therapeutic approaches that correct or ‘rewire’ disease- or patient-specific metabolic and energetic defects, using mitochondrial disorders and amyotrophic lateral sclerosis as case studies.

Lead Principal Applicant: Jochen Prehn
Co-applicants; Niamh M. Connolly
Simon Furney

Daniele Bano Division or department Bonn Organisation Deutsches Zentrum für Neurodegenerative Erkrankungen e. V.
(DZNE) in der Helmholtz-Gemeinschaft

CoEN Funding partners: SFI and Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE), Germany