Staff Profile

Qualifications

BSc (Hons) Biomedical Sciences (First Class), Newcastle University

MRes Neuroscience (Distinction), Newcastle University

PhD Neuroscience, Wellcome Centre for Mitochondrial Research, Newcastle University

Professional memberships

British Neuropathological Society (2021 - Present)

Biochemical Society (2023 - Present)

E-mit Society (2023 - Present)

Research Interests

My main research interests are in primary mitochondrial diseases characterised by severe neurological impairment. In particular, I am keen to understand the neuropathophysiological mechanisms underpinning refractory epilepsy in patients with POLG-related disease, including the paediatric onset form Alpers' syndrome. My research to date has employed post-mortem brain tissues from patients with Alpers' syndrome to delineate the specific cellular vulnerabilities contributing to neuronal hyperexcitability in POLG-related disease. In addition to performing detailed neuropathology studies, my ongoing research is also focussed on modelling the neuropathophysiological features of POLG-related mitochondrial epilepsy using novel in vitro brain slice platforms. The aim of these studies is to better understand early stages of disease pathogenesis and to identify therapeutic targets.

Funders

Great Ormond Street Hospital Charity

The Ryan Stanford Appeal

• Articles

  • Smith LA, Chen C, Lax NZ, Taylor RW, Erskine D, McFarland R. Astrocytic pathology in Alpers’ syndrome. Acta Neuropathologica Communications 2023, (11), 86.
  • Smith LA, Erskine D, Blain A, Taylor RW, McFarland R, Lax NZ. Delineating selective vulnerability of inhibitory interneurons Alpers' syndrome. Neuropathology and Applied Neurobiology 2022, 48(6), e12833.

• Review

  • Olkhova EA, Smith LA, Bradshaw C, Gorman GS, Erskine D, Ng YS. Neurological Phenotypes in Mouse Models of Mitochondrial Disease and Relevance to Human Neuropathology. International Journal of Molecular Sciences 2023, 24(11), 9698.