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: Elizabeth Kriscenski-Perry, Attila D Kovács, David A Pearce
• Journal: Journal of child neurology (J Child Neurol) Vol. 28 Issue 9 Pg. 1137-41 (Sep 2013) ISSN: 1708-8283 [Electronic] United States
• PMID: 23838029 (Publication Type: Journal Article, Research Support, N.I.H., Extramural)

Chemical References

• Cln6 protein, mouse
• Cln8 protein, mouse
• Membrane Proteins

Topics

• Animals
• Disease Models, Animal
• Growth Disorders (etiology, genetics, physiopathology)
• Membrane Proteins (genetics)
• Mice
• Neuronal Ceroid-Lipofuscinoses (complications, genetics, physiopathology)
• Seizures (etiology, genetics, physiopathology)