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A recurrent GARS mutation causes distal hereditary motor neuropathy
Journal article   Peer reviewed

A recurrent GARS mutation causes distal hereditary motor neuropathy

Diana C Lee, Rebecca Meyer-Schuman, Chelsea Bacon, Michael E Shy, Anthony Antonellis and Steven S Scherer
Journal of the peripheral nervous system, Vol.24(4), pp.320-323
12/2019
DOI: 10.1111/jns.12353
PMCID: PMC6910989
PMID: 31628756

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Abstract

We found a p.Gly327Arg mutation in GARS in two unrelated women, both of whom had a similar phenotype - motor weakness that began in late childhood, distal weakness in the arms and legs, a motor greater than sensory neuropathy with slowing of motor and not sensory conduction velocities. A de novo mutation was proven in one patient and suspected in the other. The p.Gly327Arg GARS variant did not support yeast growth in a complementation assay, showing that this variant severely impairs protein function. Thus, the p.Gly327Arg GARS mutation causes a distal motor neuropathy.
HMN aminoacyl transferase CMT Charcot-Marie-Tooth disease

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