Nadia L. Mitchell

A new large animal model of CLN5 neuronal ceroid lipofuscinosis in Borderdale sheep is caused by a nucleotide substitution at a consensus splice site (c.571+1G>A) leading to excision of exon 3.

Lentiviral-mediated gene transfer to the sheep brain: implications for gene therapy in Batten disease.

Inhibition of storage pathology in prenatal CLN5-deficient sheep neural cultures by lentiviral gene therapy.

Translational neurophysiology in sheep: measuring sleep and neurological dysfunction in CLN5 Batten disease affected sheep.

Recent studies of ovine neuronal ceroid lipofuscinoses from BARN, the Batten Animal Research Network.

Rapid and Progressive Regional Brain Atrophy in CLN6 Batten Disease Affected Sheep Measured with Longitudinal Magnetic Resonance Imaging.

Molecular neuropathology of the synapse in sheep with CLN5 Batten disease.

An EEG Investigation of Sleep Homeostasis in Healthy and CLN5 Batten Disease Affected Sheep.

Characterisation of early changes in ovine CLN5 and CLN6 Batten disease neural cultures for the rapid screening of therapeutics.

Practical implications of motion correction with motion insensitive radial k-space acquisitions in MRI.

Longitudinal In Vivo Monitoring of the CNS Demonstrates the Efficacy of Gene Therapy in a Sheep Model of CLN5 Batten Disease.

Computed tomography provides enhanced techniques for longitudinal monitoring of progressive intracranial volume loss associated with regional neurodegeneration in ovine neuronal ceroid lipofuscinoses.

The development of brain magnetic resonance approaches in large animal models for preclinical research.

Intravitreal gene therapy protects against retinal dysfunction and degeneration in sheep with CLN5 Batten disease.

Electroretinography data from ovine models of CLN5 and CLN6 neuronal ceroid lipofuscinoses.

Ocular therapies for neuronal ceroid lipofuscinoses: more than meets the eye.

The Translational Benefits of Sheep as Large Animal Models of Human Neurological Disorders.

Natural history of retinal degeneration in ovine models of CLN5 and CLN6 neuronal ceroid lipofuscinoses.