Profile
Overview
Torrance, CA 90502
Phone: 424-571-7754
Fax: 424-571-7556
- College: BSc (Hons) Anatomy & Cell Biology, University of Sheffield UK (1986); PhD Neuroanatomy, University of Bristol, UK (1990)
- Postdoctoral Fellowships: University of Cambridge, UK (1990-1993), Max-Planck-Institute for Psychiatry, Martinsried, Germany (1993-1996), University of California, San Francisco (1996-1998), Stanford University (1998-2000).
Macauley SL, Roberts MS, Wong AMS, McSloy F, Reddy AS, Cooper JD, Sands MS (2012). Synergistic effects of CNS-directed gene therapy and bone marrow transplantation in the murine model of infantile neuronal ceroid lipofuscinosis. Ann Neurol. 71, 797-804.
Groh J, Kühl TG, Duckett S, Ip CW, Mirza M, Langmann T, Nelvagal HR, Sri S, Cooper JD, Martini R (2013). Immune cells mediate neuronal damage in a mouse model of Infantile Neuronal Ceroid Lipofuscinosis, Brain 136, 1083-101.
Macauley SL, Wong AMS, Shyng C, Augner Persson D, Dearborn J, Pearse Y, Roberts M, Fowler SC, Cooper JD, Watterson DM, Sands MS (2014). An anti-neuroinflammatory that targets dysregulated glia enhances the efficacy of CNS-directed gene therapy in murine infantile neuronal ceroid lipofuscinosis. J Neurosci 34(39):13077-82
Lu JY, Nelvagal HR, Wang L, Birnbaum SG, Cooper JD, Hofmann SL (2015). Intrathecal enzyme replacement therapy improves motor function and survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis. Molecular genetics and metabolism. 116(1-2):98-105.
Vuillemenot BR, Kennedy D, Cooper JD, Wong AMS, Sri S, Doeleman T, Katz ML, Coates JR , Johnson GC, Reed RP, Adams EL, Butt MT, Musson DG, Henshaw J, Keve S, Cahayag R, Tsuruda LS, O’Neill CA (2015). Nonclinical evaluation of CNS-administered TPP1 enzyme replacement in canine CLN2 neuronal ceroid lipofuscinosis. Mol Genet Metab 114, 281-93.
Cooper JD, Tarczyluk MA, Nelvagal HR. Towards a new understanding of NCL pathogenesis. Biochimica et biophysica acta. 2015; 1852(10 Pt B):2256-61.
J on Cooper has recently been recruited to LA BioMed from King’s College London, where he was Professor of Experimental Pathology and Head of Graduate Studies (Research). His Pediatric Storage Disorders Laboratory (PDSL) was founded in 2000 after his previous spell in California, when he was a postdoc at UCSF and Stanford. Jon is a leading international authority on the neuropathology of Batten disease or Neuronal Ceroid Lipofuscinosis (NCLs), and other lysosomal storage disorders.
By characterizing mouse and larger animal models of NCL, and human cases, his lab has made several key discoveries in these disorders including the selective nature of neuron loss, and the importance of early glial activation in rather than the accumulation of storage material these disorders. More recently, his work has extended to the effects of disease outside the brain including the spinal cord, peripheral and autonomic nervous systems, and other organs including the heart.
Together with colleagues from around the world his lab is also involved in the pre-clinical testing of a range of therapeutic strategies including enzyme replacement, gene therapy, neural stem cell transplantation and small molecule drugs, several of which have reached clinical trials.