Spinal motor neuron protein supersaturation patterns are associated with inclusion body formation in ALS

Research Highlight

Molecular origins of ALS

© Science Photo Library – KTSDESIGN/Brand X Pictures/Getty

© Science Photo Library – KTSDESIGN/Brand X Pictures/Getty

Protein clumps associated with ALS arise from precarious protein levels in motor neurons found in the spinal cord.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that leads to muscle failure and is eventually fatal. It has been linked to clumps of apparently unrelated misfolded proteins, including SOD1, TDP-43, and FUS.

Now, a team including researchers from the University of Wollongong have created a list of all proteins found in ALS clumps and studied their concentrations in nerve cells. They found that, under normal physiological conditions, these otherwise unrelated proteins all exceed a critical concentration level in spinal motor neurons – nerve cells that control movement – that makes them more likely to cluster if the body can no longer balance protein levels as a result of ageing or disease.

The team generated a database that links protein supersaturation to cell dysfunction in ALS to provide further insight into the molecular origins of this disease.

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  1. PNAS 114, E3935–E3943 (2017). doi: 10.1073/pnas.1613854114
Institutions Share
University of Cambridge, United Kingdom (UK) 0.49
University of Wollongong (UOW), Australia 0.18
Northwestern University (NU), United States of America (USA) 0.10
University of New South Wales (UNSW Sydney), Australia 0.07
Centre for Genomic Regulation (CRG), BIST, Spain 0.06
Pompeu Fabra University (UPF), Spain 0.06
Columbia University Irving Medical Center (CUIMC), CU, United States of America (USA) 0.02
Catalan Institution for Research and Advanced Studies (ICREA), Spain 0.02