Greatly influenced by Mr. Spock, perhaps it was a logical decision for Jon Cooper to become a scientist. Another great influence was Doctor Who, and although he has (so far) failed to achieve time travel, he has instead worked at several institutions in Europe and the US. The latest stage of this journey has just brought him and his family across country from Southern California to St Louis, in order to start a new position in Pediatrics.
This journey originally started in Southampton in the UK, a place best known as the home port of the Titanic. Less well known is that it was also the starting point for the Pilgrim Fathers voyage in the Mayflower, before it ran into a storm and put into Plymouth for repairs. Growing up in Southampton, Jon developed a love for photography and the cinema, that continue to this day. Less wisely he became helplessly devoted to Southampton FC, and fooled by Saints winning the FA Cup in 1976, he is still waiting optimistically for a repeat that looks increasingly unlikely.
Jon’s academic training started with a degree in Anatomy at the University of Sheffield. This was a formative experience, which included spells working as a mortuary assistant for the forensic service, but much more importantly this was also where he met future wife, Alison. Fascinated with the structure of the brain, Jon then moved to Bristol for PhD studies into the pathways that control eye movements using classic neuroanatomic tracing. This was an excellent training in brain anatomy, but like many new postdocs with something to prove to their thesis advisors, Jon changed fields and began studying neurodegeneration.
Initially, working with Michael Sofroniew at the University of Cambridge, this focused on the role of neurotrophins (like nerve growth factor) in the cholinergic basal forebrain. This allowed Jon to develop a love for quantitative microscopy, especially stereology, as an efficient and unbiased means to assess the impact of lesions upon the brain. Subsequently, he moved to Hans Thoenen’s lab at the Max-Planck-Institute for Psychiatry in Munich, Germany. This not only provided the chance to add molecular biology and making genetically modified mice to his skills, but also to appreciate the advantages of living abroad, not to mention the biergartens of Bavaria!
At this point family and science life took a significant and very positive change, when oldest son Isaac was born in 1996. Jon spent six months as a ‘stay at home dad’, and during this time had the chance to move to Bill Mobley’s lab at UCSF to start working on the rare pediatric disorders that have been his career ever since. Key to this career choice was meeting the Aurelio family, whose daughter Natalie had the CLN2 form of Neuronal Ceroid Lipofuscinosis (NCLs or Batten disease). Indeed, her picture remains close to Jon’s computer to this day. Arriving in San Francisco, also gave Jon a second focus for sporting disappointments. Despite the Giants suffering their worst season ever, Jon became hooked on baseball, an affliction that continues to this day despite the Giants’ only occasional flirtations with the post season, and sometimes even World Series success.
Like many other pediatric neurodegenerative disorders, very little was known initially about how the brain was affected in the NCLs. Starting at UCSF and continuing when the lab moved to Stanford, Jon began systematically analyzing the first available mouse models of NCL and identified the neuron populations that are most vulnerable. After returning to the UK for a faculty appointment at the Institute of Psychiatry, King’s College London, he established the Pediatric Storage Disorders lab (PSDL). Time in London also saw the family grow, with the addition of Samuel and Joseph in the early 2000s, and Alison’s career in social pediatrics also flourished. In addition to his research, Jon became increasingly committed to student welfare, and eventually ran a Graduate Program for several hundred students across three campuses.
Meanwhile, the PSDL became the leading international center for studying the neuropathology of the NCLs and other related lysosomal disorders. The lab has identified most of the key features of these disorders, in both mouse and larger animal models, comparing these findings to human autopsy samples. This has not only given us a much greater understanding of these diseases, but also provided landmarks for judging the efficacy of a range of different experimental therapies, including enzyme replacement, gene therapy, neural stem cell grafts and a variety of small molecule drugs. This pre-clinical work has resulted in several clinical trials, and cumulated in the recent FDA approval of enzyme replacement with Brineura as a treatment for CLN2 disease, the same form of NCL that Natalie Aurelio had.
In the summer of 2016 Jon returned to California to take up a new position at Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center. Surprisingly, this wasn’t entirely due to the chaos that is Brexit, or the lure of the Torrance microbrewing revolution. Instead the motivation to move was the chance to build a group of investigators, devoted to better understanding and devising therapies for lysosomal storage disorders. Working closely together with Patricia Dickson, has increasingly seen the application of principles learnt from the NCLs to different forms of mucopolysaccharidoses (MPS).
The opportunity to bring this work to Wash U, where there is already great expertise in these disorders, is a very exciting one. Jon has worked closely with Mark Sands (Medicine) for over 20 years, and already knows something of the great potential for collaboration on campus, and he has enjoyed his welcome in the department. The family made their move to St Louis an adventure, with a 10-day road trip taking in the wonders of the Valley of Fire, Monument Valley, and Mesa Verde, the Woody Guthrie Center and selected parts of Route 66 (see pictures taken by Jon). They are now settling into their new home, and life in St Louis, despite further ‘adventures’ with bats.