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Fig. 7 | Acta Neuropathologica Communications

Fig. 7

From: Cell-specific vulnerability to metabolic failure: the crucial role of parvalbumin expressing neurons in creatine transporter deficiency

Fig. 7

Behavioral, physiological and EEG characterization of PV::CrTfl animals. a Percentage of spontaneous alternation in the Y-maze test, showing a poorer performance in PV::CrTfl−/y (c-KO, n = 8) mice compared to PV::CrTfl+/y littermates (c-WT, n = 9; t-test, p < 0.01). b A significantly lower discrimination index was found in PV::CrTfl−/y mice with respect to PV::CrTfl+/y littermates (n = 5 for both groups; t-test p < 0.01). c Total distance moved in the open field arena did not differ between PV::CrTfl−/y and PV::CrTfl+/y mice (n = 5 for both groups; Mann–Whitney test, p = 0.69). d PV::CrTfl−/y and PV::CrTfl+/y animals spent a comparable amount of time in the center of the open field arena (t-test, p = 0.824). e Representative images of IOS imaging in the visual cortex of PV::CrTfl+/y (top, n = 13) and PV::CrTfl−/y mice (bottom, n = 12). Dark areas represent active portions of brain tissue. The look-up-table is also shown. Scale bar: 1.8 mm. L, lateral; A, anterior. f Quantification of IOS imaging showed an increased amplitude of the hemodynamic response in mutant animals (t-test: p < 0.05). g Effect of kainic acid (KA) treatment at the behavioral level. Circles represent the maximum seizure rating score of individual mice over a period of 1 h after KA administration. PV::CrTfl−/y mice displayed a higher Racine score compared to wild-type littermates (n = 18 for both groups; t-test, p < 0.05). h–k Severity of the epileptic phenotype in response to KA at the electrophysiological level. PV::CrTfl−/y mice have a lower latency to the first seizure (h, t-test, p < 0.05), and increased frequency (i, t-test, p < 0.05) and duration (j, t-test, p < 0.01) of seizure events with respect to PV::CrTfl+/y animals. For PV::CrTfl+/y animals not presenting seizures during the 1 h of monitoring, the observation was extended until the occurrence of the first electrographical burst to provide a latency value. Circles represent single data values. Relative percentage of tonic, clonic and tonic–clonic seizures in PV::CrTfl+/y and PV::CrTfl−/y (k) indicates that seizure severity is more pronounced i PV::CrTfl−/y animals (χ2 test; p < 0.001). *p < 0.05, **p < 0.01, ns: not significant. Data are expressed as mean ± SEM

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