Early Changes in Skeletal Muscle of Young C22 Mice, A Model of Charcot-Marie-Tooth 1A.

ABSTRACT:

Background

The C22 mouse is a Charcot-Marie-Tooth 1A transgenic model with minimal axonal loss.

Objective

To analyse early skeletal muscle changes resulting from this dysmyelinating neuropathy.

Methods

Histology of tibialis anterior muscles of C22 mice and wild type litter mate controls for morphometric analysis and (immuno-)histochemistry for known denervation markers and candidate proteins identified by representational difference analysis (RDA) based on mRNA from the same muscles; quantitative PCR and Western blotting for confirmation of RDA findings.

Results

At age 10 days, morphometry was not different between groups, while at 21 days, C22 showed significantly more small diameter fibres, indicating the onset of atrophy at an age when weakness becomes detec

SUBMITTER: Deres F 

PROVIDER: S-EPMC8673495 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature