Assessment of axonal regeneration in Gjb1/CMT1X mouse model


Alsu Shagieva

Document Type


Publication Date

Summer 2022



JAX Location

In: Student Reports, Summer 2022, The Jackson Laboratory


Charcot-Marie-Tooth (CMT) disease is a peripheral neuropathy that causes muscle weakness and loss of sensation in distal limbs with an estimated frequency of 0.04. One of the CMT forms, X-linked, (CMT1X) is caused by mutations in GJB1. It is known that while in CMT1X demyelinating occurs, increase in axonal clusters also takes place. Those clusters are believed to form due to axonal regeneration. However, it has not been shown yet that those axons in bundles in fact regenerate. SCG10 (STMN2) labeling, a marker of regenerating axons, was performed on Gjb1/CMT1X mice to assess whether axon clusters in Gjb1/CMT1X mice model are formed due to axonal regeneration. While we showed that STMN2 indeed labels regenerating axons after nerve crush, the STMN2 signal in GJB1/CMT1X was similar to WT mice, suggesting that there’s a time sensitive component in detecting regeneration with STMN2 staining.

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