title: Seizure susceptibility, phenotype, and resultant growth delay in the nclf and mnd mouse models of neuronal ceroid lipofuscinoses.
abstract: We examined flurothyl gas-induced seizure latencies and phenotype in 2 mouse models of neuronal ceroid lipofuscinoses: the nclf (Cln6 mutant) variant late-infantile model and the mnd (Cln8 mutant) Northern epilepsy model. Mnd mice on postnatal days 35 to 42 had increased latency to loss of posture compared with wild-type controls. Nclf, mnd, and wild-type mice on postnatal days 21 days to 25 displayed similar latency profiles during repeated seizure induction (kindling) and retesting; seizure phenotypes were different, however. Kindled wild-type mice reexposed to flurothyl after a 28-day recovery displayed brainstem generalized seizures exclusively. Neuronal ceroid lipofuscinoses mutants demonstrated a lack of brainstem seizures at retesting after 28 days. Repeated induction of generalized seizures delayed weight gain in both nclf and mnd mice compared with wild-type mice. These and our previous results suggest that abnormal seizure-related neuronal connectivity and/or plasticity are shared characteristics of the neuronal ceroid lipofuscinoses.
authors.affiliation: Center for Neural Development and Disease, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Kriscenski-Perry
authors.forename: Attila D
authors.initials: AD
authors.lastname: Kovács
authors.forename: David A
authors.initials: DA
authors.lastname: Pearce
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D006130:Growth Disorders / Q000209:etiology* / Q000235:genetics / Q000503:physiopathology; D008565:Membrane Proteins / Q000235:genetics; D051379:Mice; D009472:Neuronal Ceroid-Lipofuscinoses / Q000150:complications* / Q000235:genetics / Q000503:physiopathology; D012640:Seizures / Q000209:etiology* / Q000235:genetics / Q000503:physiopathology
chemical_list: C486722:Cln6 protein, mouse; C498493:Cln8 protein, mouse; D008565:Membrane Proteins
keywords: Batten disease; epilepsy; flurothyl; neuronal ceroid lipofuscinoses; seizure induction latencies