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Indeed, significant accumulation of autofluorescent storage material, the ultrastructure of storage material, increased astrocytosis and microglial activation, as well as myelination defects seen in Cln1/5 dko mice resemble the more severe pathological features of Cln1 ko mice, whereas the onset of neuron loss is similar to that observed in Cln5 ko mice.
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At 8 dpf, ER swelling is severe; other pathological features include disrupted bile canaliculi, altered cytoplasmic matrix and accumulation of large lysosomes.
Infection with the highest dose of MCMV in Group D resulted in the most severe lung pathological features and interstitial pneumonitis-like pathological changes at 14 d and 21 d postinfection.
Recently, we have shown that the main pathological features of severe PAM are reproduced in a mouse model [22] and Megnekou et al. [23] evidenced that pregnant mice accumulated specific antibodies against recrudescent parasite.
Together, these data strongly suggest that fetuses from infected mothers suffer IUGR and have decreased viability due to placenta blood flow impairments, recapitulating pathological features of severe malaria manifestations typically observed in pregnant women from low malaria transmission regions [4].
These mice develop a severe arthritis that shares some pathological features with human RA.
Overall, the clinical and pathological features were severe, but did not fall outside the usual disease spectrum.
A previous study on the homozygous case described the clinical and pathological features as severe, but not completely outside of the usual disease spectrum [ 12].
Recently, necrotizing autoimmune myopathies (NAM) have been defined as a distinct group of severe acquired myopathies, characterized by pathological features of prominent myofiber necrosis without significant inflammation [ 1].
The phenomenon of sequestration, first described in the late 19th Century by the Italian pathologists Marchiafava and Bignami (1892), has been associated with pathological features of severe malaria (see below) and the molecular events are largely ascribed to PfEMP1, although it is possible that other parasite and host-derived molecules may participate in this important interaction.
One of the pathological features of the severe dystrophic phenotype is a continuous cycle of degeneration and regeneration that is exacerbated in mdx mice by increased levels of IL-6.
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