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.