IN MEMORIAM George Karpati (1934-2009)

George Karpati, the Isaac Walton Killam Professor of contributed in a significant way to the understanding of their Neurology at McGill University and a world renowned specialist underlying pathophysiology. in neuromuscular disorders died suddenly on February 6, 2009 He discovered the surface plasma membrane localization of during a working dinner at the McGill Faculty Club. dystrophin, the defective gene product in Duchenne Muscular He was born in Debrecen in North Eastern Hungary and Dystrophy. This discovery was an important step in the design of towards the end of the second world war he was deported with therapeutic strategies for muscular dystrophies, to which he his parents to one of the infamous work camps to which devoted much of his career with pioneering work in myoblast Hungarian Jews were then sent. His father was sent further to the transfer, stem cell therapy, viral mediated gene transfer and extermination camp at Bergen Belsen. George never talked about upregulation strategies for homologous proteins. his early life, except on the He had numerous disciples, who insistence of his sons. eventually went on to dis- He left Hungary with his tinguished careers in the field of mother at the time of the muscle pathology, neuromuscular Hungarian revolution in 1956. neurology and genetics. (Calvin He completed his medical Melmed, Guy Rouleau, Agnes education at Dalhousie Jani, Joseph Jossiphov, Bassem University and then joined the Yamut, Jordi Casademont, Morris Neurological Hospital. Danon, Boaz Weller, Johnny He did postgraduate work with Huard, Gyula Acsadi, Giovanna King Engel at the NIH and Pari, Michel Melanson, Maria returned to the MNI as an Molnar, Rita Horvath, Hanns Assistant Professor in 1967. Lochmuller, Wendy Johnston, George Karpati had a boundless Michelle Mezei, Antal Szabo, curiosity and fascination with Dubravka Dodig, Amelie Nadeau, scientific investigation. He was Erin O’Ferrall, amongst many). very widely read, an excellent He was happily married to Shira diagnostician and a superb Tannor and they had two sons teacher. Muscle pathology was Adam and Joshua to whom he was his overwhelming passion, which extremely devoted. George led to the publication of an Karpati was actively involved in authoritative text written with the medical and scientific life Stirling Carpenter, his partner in of the Montreal Neurological the study of muscle diseases and Hospital and Institute and the his friend. With the neurochemist University. His expertise was Leon Wolfe, Stirling Carpenter, widely recognized. the geneticist Eva Andermann and the senior author of this He was an Officer of the Order of and a Chevalier of obituary, he studied a panoply of progressive neurological the Order of . He was a fellow of the Royal Society of disorders, including the cerebral lipidoses, white matter Canada and received the Wilder Penfield Award of the disorders and storage diseases. government of Quebec, the Governor General’s anniversary He established a neuromuscular research group at the MNI to medal, the distinguished scientist award from the Canadian tackle the biology of neuromuscular diseases from different Society of Clinical Investigation, Lifetime Achievement Awards perspectives and recruited outstanding people in their respective from the Muscular Dystrophy Association Canada, World field of cell biology (Heather Durham, Josephine Nalbantoglu), Federation of Neurology and the Montreal Neurological biochemistry (Paul Holland), molecular biology (Ken Hastings), Institute, as well as honorary doctoral degrees from the physiology (Basil Petrof, Tanja Taivassalo), genetics (Renald Universities of Marseilles and of Debrecen. He was an honorary Gilbert), mitochondrial diseases (Eric Shoubridge) and member of the Hungarian Academy of sciences and of the neuromuscular neurology (Daniel Gendron, Angela Genge), American Neurological Association. rendering the MNI one of North America’s foremost centers for His death is a heavy loss to our entire community and to the neuromuscular medicine. world of muscle research. He was one of the first to describe now well recognized neuromuscular entities such as inclusion body myositis, polyglucosan body disease, giant axonal neuropathy, adult onset Michael Sinnreich, Frederick Andermann acid maltase deficiency, myosin depletion myopathy and has Montreal, Quebec

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