Lambert-Smith, Isabella Dr.

Selected Publications

• Journal Article

  Year	Title
  2022	Proteostasis impairment and ALS Published In Progress in Biophysics and Molecular Biology
  2020	The pivotal role of ubiquitin-activating enzyme E1 (UBA1) in neuronal health and neurodegeneration Published In International Journal of Biochemistry and Cell Biology
  2018	SOD1A4V aggregation alters ubiquitin homeostasis in a cell model of ALS Published In Journal of Cell Science
  2017	Addition of exogenous SOD1 aggregates causes TDP-43 mislocalisation and aggregation Published In Cell Stress and Chaperones
  2017	Spinal motor neuron protein supersaturation patterns are associated with inclusion body formation in ALS Published In Proceedings of the National Academy of Sciences of USA
  2015	Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions Published In Scientific Reports
  2014	ALS-associated mutants of SOD1, TDP-43, FUS, C9ORF72 and UBQLN2 disrupt proteostasis in neuronal cell culture Published In Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
  2014	Amilial amyotrophic lateral sclerosis-associated proteins form aggregates via distinct pathways in cells Published In Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration