Identifying metabolic impairments in motor neuron disease (MND) and testing possible metabolic treatments

Dr Tefera’s research is currently centred on understanding mechanisms of metabolic dysfunctions in MND and investigating metabolic treatments. MND is a fatal neurodegenerative disorder characterized by progressive loss of motor neurons in the brain and the spinal cord which finally leads to skeletal muscle atrophy, paralysis and death. There is no cure for MND and only two drugs are approved for use in ALS. Although, several hypotheses have been forwarded, the exact mechanisms of disease progression in this disease are not comprehensively understood. Growing body of evidence shows alterations in energy metabolism in animal models of MND as well as people living with MND. Dr Tefera investigates how CNS and skeletal muscle energy metabolism dysfunction could contribute to the onset and progression of the disease. He also explores if modifying specific metabolic pathways could be used as a therapeutic strategy to slow disease progression and prolong survival in MND.

Dr Tesfaye Tefera is highly interested in understanding how cells utilize energy to maintain normal physiological function and how alterations in energy production and utilization pathways contribute to neurodegenerative diseases. He aims to decipher specific impairments in metabolic pathways that can be targeted by novel compounds.

Dr Tefera holds a PhD from The University of Queensland (Australia), a master degree in Neuroscience from Norwegian University of Science and Technology (Norway) and a bachelor degree in Pharmacy from Addis Ababa University (Ethiopia). His PhD work was focused on characterizing energy metabolism defects in CNS tissues of the hSOD1G93A mouse model of MND. Using a combination of analytical techniques including high – performance liquid chromatography (HPLC), liquid chromatography - tandem mass spectrometry (LC-MS/MS), 1H and 13C nuclear magnetic resonance (NMR) spectroscopy, he was able to identify specific metabolic impairments particularly related to glucose metabolism via glycolysis and pentose phosphate pathways as well as amino-acid neurotransmitters in cortical and spinal tissues of the hSOD1G93A mouse model of MND. He has also investigated neuroprotective and disease-modifying potential of a metabolic treatment in hSOD1G93A mice.

Dr Tefera has won competitive scholarships to undertake his PhD and master studies and has obtained various travel awards. He is now a postdoctoral research fellow at the Australian Institute for Bioengineering and Nanotechnology as well as a member of International Society of Neurochemistry.

 

Funding

Dr Tefera was supported by the University of Queensland International Scholarship to complete his PhD studies.

 

Key Publications

  1. Tefera, T.W., Bartlett K., Tran S.T., Hodson M.P., Borges K. Impaired pentose phosphate pathway in the spinal cord of the hSOD1G93A mouse model of Amyotrophic Lateral Sclerosis. Mol. Neurobiol. 2019. https://doi.org/10.1007/s12035-019-1485-6
  2. Tefera, T.W., Borges K. Neuronal glucose metabolism is impaired while astrocytic TCA cycling is unaffected in the hSOD1G93A mouse model of amyotrophic lateral sclerosis. J. Cereb. Blood Flow Metab. 2018. doi: 10.1177/0271678X18764775.
  3. Tefera, T.W., Borges K. Metabolic Dysfunctions in Amyotrophic Lateral Sclerosis Pathogenesis and Potential Metabolic Treatments. Front. Neurosci. 2017: 10:611. doi: 10.3389/fnins.2016.00611.
  4. Tefera, T.W., Tan K., McDonald T.S., Borges K. Alternative fuels in epilepsy and Amyotrophic lateral sclerosis. Neurochem Res 2016: 42(6):1610-1620. doi: 10.1007/s11064-016-2106-7.
  5. Tefera T.W., Wong Y, Barkl-Luke ME, Ngo ST, Thomas NK, McDonald TS, et al. Triheptanoin Protects Motor Neurons and Delays the Onset of Motor Symptoms in a Mouse Model of Amyotrophic Lateral Sclerosis. PLoS ONE 2016: 11(8): e0161816. doi: 10.1371/journal.pone.0161816.

 

Featured projects Duration
Metabolism and skeletal muscle pathology in MND
20152018