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This research aims to investigate how copper restriction affects axonal degeneration in a specific mouse model of hereditary motor neuropathy.

June 8, 2026MetallomicsOpen Access

Copper restriction unmasks axonal degeneration in a mouse model of X-linked hereditary motor neuropathy

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Randomized trial demonstrates axonal loss in a copper-restricted mouse model of hereditary motor neuropathy, suggesting critical copper roles.

This research aims to investigate how copper restriction affects axonal degeneration in a specific mouse model of hereditary motor neuropathy.
Crossed Atp7aT985I mice with Ctr1+/- mice to genetically restrict copper availability.
Performed behavioral testing and histopathological assessments at six months.
Conducted tandem mass tag-based proteomics on sciatic nerve samples.
Atp7aT985I/Ctr1+/- males showed significantly reduced copper levels in both CNS and PNS.
Mild motor deficits and axonal loss were observed, especially in small-caliber fibers within copper-restricted mice.
Significant proteomic changes related to energy metabolism, cytoskeletal integrity, and cellular stress were identified.

Perez-Siles et al. (2026) studied this question.

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Randomized trial demonstrates axonal loss in a copper-restricted mouse model of hereditary motor neuropathy, suggesting critical copper roles.