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The newspaper of The Johns Hopkins University January 29, 2007 | Vol. 36 No. 19
 
Hugo Moser, World-Renowned for Fight Against ALD, Dies at 82

Hugo Moser

Researcher portrayed in movie 'Lorenzo's Oil' leaves legacy of hope

Staff
Kennedy Krieger Institute

Hugo W. Moser, recognized throughout the world for his research on genetic disorders that affect nervous system function in children, died on Jan. 20 of complications after recent vascular surgery, which followed successful October surgery for pancreatic cancer. He leaves behind a legacy of advancing the science surrounding some of the most complex genetic disorders, particularly the disease adrenoleukodystrophy, or ALD, as profiled in the 1992 film Lorenzo's Oil.

Moser, who was 82, served as president of the Kennedy Krieger Institute from 1976 to 1988 and as director of its Neurogenetics Research Center from 1995 until his death. At Johns Hopkins, he held the title University Professor of Neurology and Pediatrics.

"Hugo Moser's passing is a profound loss for the field of neurogenetics, the international adrenoleukodystrophy community and all who had the privilege of working with this great man," said Gary Goldstein, president of the Kennedy Krieger Institute. "As anyone who knew him could attest, he was far more than a dedicated faculty member at Kennedy Krieger; he was a friend and mentor to us all. His sole motivation during his 30-plus-year tenure at the Kennedy Krieger Institute was to help those suffering. Amid the many appointments he held, papers he published, studies he led and awards he received, he never wavered from this commitment."

As a neurologist specializing in genetic disorders that involve the part of the cell called the peroxisome, Moser helped to identify the characteristic biochemical abnormalities and the gene mutations that cause each of the 15 peroxisomal disorders. However, he is best known for his work in the most common of these disorders, ALD.

In ALD, Moser established methods of early diagnosis, counseling and worldwide programs to evaluate methods of therapy, including diet, pharmacological agents and transplantation. Most recently, his landmark study of the preventive effects of Lorenzo's Oil proved that the oil prevents the onset of disease symptoms for the vast majority of children suffering from this debilitating disorder.

Augusto Odone, developer of Lorenzo's Oil and father of the boy who made the disease famous, said, "Without Hugo's tireless commitment to studying the efficacy of Lorenzo's Oil, we would still be lacking the scientific validation that has proven the oil prevents the onset of ALD symptoms. Through this journey, I found not only a brilliant partner in ALD research but a dear friend whose support has been invaluable to our family all these years." Odone is now president of the Myelin Project, an organization that works to accelerate research on myelin repair. Lorenzo, diagnosed when he was 5, is now 28.

Even before Lorenzo's Oil had been shown to prevent the onset of symptoms, Moser and his wife and research partner, Ann Boody Moser, were moving full steam ahead with developing a screening technique that would allow ALD to be detected at birth. In July 2006, a published study outlined a technique for an ALD screening to be "piggybacked" onto states' existing newborn screening programs. Broader testing of the technique is presently under way at the Kennedy Krieger Institute, led by Ann Moser.

Paula Brazeal, president of the United Leukodystrophy Foundation and a longtime friend of the Mosers, said, "Hugo and Ann have been relentless in their search for a screening technique that can be readily applied to states' existing newborn screening programs. Through this most recent discovery, the heavy lifting has been done. Now, it is just a matter of carrying on the torch and seeing this method through. We all sadly mourn the great loss of Hugo, founding father of our own organization, but we will continue to carry on his legacy by advancing his research."

Born in Bern, Switzerland, Hugo Wolfgang Moser spent his early childhood in Berlin, Germany, where his father was an art dealer and his mother an actress. His family, including his brother, Henry, fled Nazi Germany in 1933 and lived in Heemstede, Holland. In February 1940, they crossed France, Italy and Spain and finally embarked to Cuba before obtaining visas to immigrate to New York City.

Moser's medical career began in the late 1940s, when he received his degree from the College of Physicians and Surgeons at Columbia University and went to work as an intern at the Columbia-Presbyterian Medical Center. In the early 1950s, he served in the Korean War as a medical officer in the U.S. Army. Returning from Korea and wanting to prepare himself for a life of research, Moser received a master's degree in biological chemistry from Harvard. From 1957 to 1960, he was a resident in neurology at Massachusetts General Hospital, where he later served as professor of neurology. Following his extensive training in the field of neurology, Moser began researching brain lipids and, with the guidance of his mentors, became interested in leukodystrophies. It was in his first lab that he met Ann, his wife of more than 40 years.

In 1976, Moser, along with other prominent scientists in the field, moved to Baltimore and the Kennedy Krieger Institute, where they began to work specifically on ALD. Together, the Mosers developed the first diagnostic test for ALD, and today's treatments, including bone marrow transplant and Lorenzo's Oil therapy, were tested in his famous lab.

Moser's career spanned more than 50 years, and he continued to work into the nights up until a few months ago, when his health began to fail. He remained extremely active throughout the ALD community, serving on the boards of the United Leukodystrophy Foundation, the Myelin Project and the Montel Williams MS Foundation. Moser also participated in many professional societies and editorial boards, and at the time of his death was on the board of directors of the International Child Neurology Society. His many accomplishments earned him wide international recognition and numerous honors and awards, including his October 2006 election to the rank of fellow of the American Association for the Advancement of Science, honoring his pioneering work in the field of developmental neurogenetics.

"Most people would retire at 65," KKI President Goldstein told The Baltimore Sun last week. "But the most productive part of his life happened in the past 20 years. He signed a grant application on his way to the operating room [for his surgery] last fall. He wanted to get this disease cured before he died."

Moser was also loved and known by researchers and families throughout the world as an advocate for persons with mental retardation and developmental disabilities.

Moser is survived by his partner in science and life, Ann Boody Moser, and three daughters, Tracey Schecht of Austin, Texas; Karen Levin of West Chester, Pa.; and Lauren Moser of Bethesda, Md.; and four grandsons. He is preceded in death by his brother, Henry Mason of New Orleans; his first wife, Monti Lou Brigham; and his son, Peter Brigham Moser.

A memorial service is planned for 11 a.m. Feb. 3 at the First Baptist Church in Wakefield, Mass. Plans for a memorial service in Baltimore are not yet finalized.

In accordance with Moser's lifelong commitment to the advancement of research for childhood neurological disorders, especially X-linked adrenoleukodystrophy, donations in his memory can be made to Peroxisomal Research, Neurogenetics Unit at the Kennedy Krieger Institute, 707 N. Broadway, Baltimore, MD 21205, or to the United Leukodystrophy Foundation, through its Web site, www.ulf.org.

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