A Mouse Model of L-2-Hydroxyglutaric Aciduria, a Disorder of Metabolite Repair
Fig 10
Sensitivity of l2hgdh-/- mice to the convulsant pentylenetetrazole.
Six l2hgdh+/+ and 6 l2hgdh-/- mice were placed in a plastic chamber (15 × 25 × 40 cm) and habituated for 10 min before intraperitoneal injection of a subconvulsive dose (40 mg/kg) of pentylenetetrazole (PTZ, Sigma-Aldrich, St. Louis, MO, USA). They were then video-tracked for 20 min and their behavior was classified and scored as follows: 0: normal; 1: immobilization; 2: facial, vibrissal and forelimb clonus (short myoclonic jerk); 3: myoclonic jerking consisting of a whole body jerk with or without irregular, bilateral forelimb movements; 4: generalized clonic seizures with kangaroo posture; 5: generalized tonic–clonic seizures with loss of posture tone; 6: death (Watanabe et al. 2013). Statistical analyses were performed using the Student t-test (p = 0.0062).