We examined whether there are gender differences in the progressive loss of functional motor units in SOD1(G93A) transgenic mice. Isometric muscle and motor unit twitch contractions were recorded in fast- and slow-twitch muscles in response to stimulation of the sciatic nerve. Using a modified motor unit number estimation technique (ITS-MUNE), we found that motor unit numbers declined rapidly from 40 to 90 days of age during the asymptomatic phase of ALS in fast- but not slow-twitch hindlimb muscles of both male and female mice. There was a corresponding decline in twitch and tetanic contractile forces of the fast-twitch muscles. Gender did not affect the progressive loss of motor units and associated decline in force production. We conclude that gender does not alter progressive, muscle-specific motor unit loss in ALS, even though gender does influence disease onset.