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Resistance exercise and creatine supplementation independently improve strength and function in patients with neuromuscular diseases, but the effectiveness of the two combined in patients with Charcot-Marie-Tooth (CMT) disease is unknown. Additionally, no study has examined gender differences in strength and activities of daily living (ADLs) in CMT patients before or after resistance training, nor has ADL performance been used for exercise prescription. Purposes. (1) To examine the effects of resistance training with and without creatine supplementation on muscle, strength and function in patients with CMT; (2) To compare effects of 12 weeks training on strength and ADLs in CMT men and women, and design an ADL-based resistance exercise prescription template. Methods. Twenty age-matched patients (11 women, 9 men, mean age 45 ± 9y) with CMT consumed 5g/d creatine or placebo while participating in resistance training for 12 weeks. Energy metabolites, muscle fiber type and size, body composition, strength, surface electromyography, timed activities of daily living, and peak oxygen consumption were measured before and after training. Results. Exercise training increased type I muscle fiber diameter (48.2 ± 14.2μm → 55.4 ± 14.8μm, p = .03), strength (p < .05), bicycle exercise duration (506s vs. 563s, p = .05) and decreased ADL times (p < .001). Training compliance was 87(±26)% with no gender differences. Absolute strength was greater in CMT men in only 4-of-10 baseline measures (p < .05), but strength normalized by lean mass was not different. After training women had 80% of normal strength in 8-of-10 exercises while men only had one. Training volumes and strength change scores were not different between genders. ADLs did not differ between genders, but improved when genders were combined (p < .05). An exercise prescription template was developed using chair-rise time to estimate starting weights for lower body, and supine-rise for upper body. Conclusions. Patients respond to resistance training with muscle fiber adaptations, and improvements in strength and function. Creatine was not beneficial. Resistance training improved strength and ADLs equally in men and women. An exercise prescription template was designed based on ADL performance.