Hugo Moser (scientist)

Hugo Moser in Korea

HugoMoser MGHNeurology 1974

Hugo Wolfgang Moser (December 4, 1924 – January 20, 2007) was a renowned American biochemist and researcher known for his significant contributions to the understanding and treatment of adrenoleukodystrophy (ALD), a genetic neurodegenerative disease. His work has had a profound impact on the field of neurology and genetic disorders, leading to advancements in diagnosis, treatment, and the potential for gene therapy in managing ALD.

Early Life and Education

Hugo Moser was born in Germany and emigrated to the United States with his family to escape the Nazi regime. He pursued his higher education in the US, earning a Bachelor of Science degree from Oberlin College and subsequently a medical degree from Harvard Medical School. His early interest in biochemistry and genetics led him to focus on metabolic disorders affecting the brain.

Career and Research

Moser's career was primarily associated with the Kennedy Krieger Institute in Baltimore, Maryland, where he served as the director of the Neurogenetics Research Center. He was also a professor of neurology and pediatrics at the Johns Hopkins School of Medicine. Throughout his career, Moser dedicated himself to the study of lipid metabolism disorders, particularly adrenoleukodystrophy.

Adrenoleukodystrophy is a condition that affects the myelin sheath, the protective covering that surrounds neurons in the brain and spinal cord, leading to progressive neurodegeneration. Moser's work was instrumental in identifying the biochemical defect associated with ALD, which involves the accumulation of very long-chain fatty acids in the body due to a mutation in the ABCD1 gene.

Contributions and Legacy

Moser's research led to the development of dietary therapy strategies, including the use of Lorenzo's oil, a mixture of glyceryl trioleate and glyceryl trierucate, which has been shown to lower the levels of very long-chain fatty acids in the body. Although not a cure, this treatment has been beneficial in delaying the onset of ALD symptoms in some patients.

Moreover, Moser played a key role in the establishment of newborn screening programs for ALD, which are crucial for early diagnosis and intervention. His advocacy for early detection and treatment has significantly improved the prognosis for many individuals with ALD.

Moser's contributions extend beyond his research on ALD. He was a mentor to many young scientists and physicians, fostering the next generation of researchers in the field of neurogenetics. His dedication to his patients and their families was evident in his compassionate approach to care and his commitment to improving their quality of life.

Death and Honors

Hugo Moser passed away on January 20, 2007, leaving behind a legacy of groundbreaking research and advocacy for patients with genetic disorders. His work has been recognized with numerous awards and honors, reflecting his impact on the fields of biochemistry, neurology, and genetics.

See Also

• Genetic disorder
• Neurodegenerative disease
• Lorenzo's Oil
• Kennedy Krieger Institute
• Johns Hopkins School of Medicine