Efficacy of dual intracerebroventricular and intravitreal gene therapy in sheep prompts the first clinical trial to treat CLN5 Batten disease.

Mutations in the gene cause the fatal, pediatric, neurodegenerative disease CLN5 neuronal ceroid lipofuscinosis. Affected children suffer progressive neuronal loss, visual failure and premature death. Presently there is no treatment.

Long-term safety and dose escalation of intracerebroventricular CLN5 gene therapy in sheep supports clinical translation for CLN5 Batten disease.

CLN5 neuronal ceroid lipofuscinosis (NCL, Batten disease) is a rare, inherited fatal neurodegenerative disorder caused by mutations in the gene. The disease is characterised by progressive neuronal loss, blindness, and premature death. There is no cure.

Cognitive and arginine metabolic correlates of temporal dysfunction in the MIA rat model of schizophrenia risk.

As a hallmark characteristic of schizophrenia, abnormal perception of time is thought to arise from cognitive impairment; however, the absence of translational models indexing this pathological relationship creates barriers to understanding the functional and biological bases of timing impairments. Here, we investigate the relationship between timing and cognition using the maternal immune activation (MIA) rat model of schizophrenia. We additionally investigate the role of prefrontal cortex L-arginine metabolism in these processes via high-performance liquid chromatography and liquid chromatography/mass spectrometry.

The instrumental role of operant paradigms in translational psychiatric research: Insights from a maternal immune activation model of schizophrenia risk.

Rigorous behavioral analysis is essential to the translation of research conducted using animal models of neuropsychiatric disease. Here we discuss the use of operant paradigms within our lab as a powerful approach for exploring the biobehavioral bases of disease in the maternal immune activation rat model of schizophrenia. We have investigated a range of disease features in schizophrenia including abnormal perception of time, cognition, learning, motivation, and internal state (psychosis), providing complex insights into brain and behavior.

Occurrence of Rat Lungworm () in Invasive Coqui Frogs () and Other Hosts in Hawaii, USA.

The rat lungworm () has emerged as an important human and animal health concern in Hawaii, US. Although the life cycle of the parasite requires both rat and gastropod hosts, other animals acting as paratenic hosts, such as frogs and centipedes, have been identified as sources of infection. We investigated the occurrence of rat lungworm infections in potential paratenic hosts in Hawaii to provide information on how they might be involved in transmission of angiostrongyliasis.

Validation of a death assay for larvae (L3) using propidium iodide in a rat model ().

Angiostrongylus cantonensis is a pathogenic nematode and the cause of neuroangiostrongyliasis, an eosinophilic meningitis more commonly known as rat lungworm disease. Transmission is thought to be primarily due to ingestion of infective third stage larvae (L3) in gastropods, on produce, or in contaminated water. The gold standard to determine the effects of physical and chemical treatments on the infectivity of A.

Maternal immune activation in rats produces temporal perception impairments in adult offspring analogous to those observed in schizophrenia.

The neurophysiology underlying temporal perception significantly overlaps with areas of dysfunction identified in schizophrenia. Patients commonly exhibit distorted temporal perception, which likely contributes to functional impairments. Thus, study of temporal perception in animal models of the disease may help to understand both cognitive and neurobiological factors involved in functional impairments in patients.