Background
Professor Cooper is a neuroscientist who has been studying the pathogenesis of Batten disease and other lysosomal storage disorders for more than 20 years.
His lab relocated to Washington University School of Medicine in St Lois, USA, in 2018 and is the leading international center for the morphological analysis of pathological changes in the NCLs.
Batten disease
His lab was the first to describe many of the key pathological findings within the CNS in the neuronal ceroid lipofuscinoses (NCLs or Batten disease). This includes the staging of disease, cell specificity and the relationship between neuron loss and glial activation.
Such data have been used as outcome measures for a variety of pre-clinical interventions, with much of this work done together with Dr. Sands.
His lab has been involved in many international collaborations to study the efficacy of different pre-clinical interventions in these disorders, leading to several clinical trials and an approved treatment for CLN2 disease. His work increasingly extends to multiple forms of mucopolysaccharidosis (MPS),
much of it in collaboration with Dr. Dickson and Dr. Sands, including pre-clinical studies in several MPS mice and MPS IIIB dogs. His lab, in collaboration with Dr. Heuckeroth, recently described enteric disease in multiple forms of NCL, which is treatable by neonatal intravenous gene therapy.
Dr Cooper’s lab generated the preliminary data for the existence of bowel transit defects and enteric neuron loss in MPS IIIA mice that forms the basis of this application.