 | Hugo W. Moser, M.D. Former Director of the Neurogenetics Research Center, Kennedy Krieger Institute In Memoriam: A Tribute to Dr. Hugo Moser (1924 - 2007) Hugo W. Moser, MD is a research scientist and Director of the Neurogenetics Research Center at the Kennedy Krieger Institute. Dr. Moser is also University Professor of Neurology and Pediatrics at Johns Hopkins University. |
Biographical Sketch:
Hugo Moser joined the faculty in 1976 as President of the Kennedy Institute. He came to the Johns Hopkins University as Professor of Neurology and Pediatrics, and in 1988 was appointed University Professor of Neurology and Pediatrics, only the ninth designee in the University's history. Since 1995 he has been the Director of the Neurogenetics Research Center at the Kennedy Krieger Institute.
Dr. Moser has many professional memberships, editorial activities, and study section and consultant appointments. His publications include over 340 referred journal articles and over 110 chapters in books and publications. His research on peroxisomal disorders has achieved international recognition, and he has been awarded several distinguished lectureships. Dr. Moser proceeds with his research work and continues to lecture at scientific meetings in the United States and abroad.
Dr. Moser has many professional memberships, editorial activities, and study section and consultant appointments. His publications include over 340 referred journal articles and over 110 chapters in books and publications. His research on peroxisomal disorders has achieved international recognition, and he has been awarded several distinguished lectureships. Dr. Moser proceeds with his research work and continues to lecture at scientific meetings in the United States and abroad.
Research Summary:
Dr. Moser has focused his research on genetic disorders that affect the nervous system function in children, particularly those that involve a part of the cell referred to as the peroxisome. There are 15 peroxisomal disorders that lead to intellectual disabilities and nervous system disabilities. The most common of these is adrenoleukodystrophy (ALD). Dr. Moser helped to identify the characteristic biochemical abnormalities and the gene mutations that cause each of these disorders. He established methods of early diagnosis and counseling and world-wide programs to evaluate methods of therapy, including diet, pharmacological agents and transplantation.
Neurogenetics Research and Peroxisomal Disease Diagnostic Laboratory This unit, which is headed by Dr. Hugo Moser, is concerned with the study, diagnosis and treatment of peroxisomal disorders, including X-linked adrenoleukodystrophy, the Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum syndrome and rhizomelic chondrodysplasia punctata. It is a highly specialized service. Patients are physician-referred and included only if preliminary clinical and laboratory studies have shown that the patient has a peroxisomal disorder. The referral base is national, and at times includes patients from other countries. Patients are seen in the KKI outpatient department or in the General Clinical Research Center at the Johns Hopkins Medical Institutions by special and prearranged appointments. The contact person is Pauline Green, R.N., at 443-923-2772. Our services include confirmation of diagnosis, precise definition of biochemical and genetic abnormalities; counseling; and the design and evaluation of new therapeutic approaches, utilizing protocols that have been approved by the Institutional Review Board and other appropriate agencies.
Neurogenetics Research and Peroxisomal Disease Diagnostic Laboratory This unit, which is headed by Dr. Hugo Moser, is concerned with the study, diagnosis and treatment of peroxisomal disorders, including X-linked adrenoleukodystrophy, the Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum syndrome and rhizomelic chondrodysplasia punctata. It is a highly specialized service. Patients are physician-referred and included only if preliminary clinical and laboratory studies have shown that the patient has a peroxisomal disorder. The referral base is national, and at times includes patients from other countries. Patients are seen in the KKI outpatient department or in the General Clinical Research Center at the Johns Hopkins Medical Institutions by special and prearranged appointments. The contact person is Pauline Green, R.N., at 443-923-2772. Our services include confirmation of diagnosis, precise definition of biochemical and genetic abnormalities; counseling; and the design and evaluation of new therapeutic approaches, utilizing protocols that have been approved by the Institutional Review Board and other appropriate agencies.
| RESEARCH PARTICIPANTS NEEDED: | Adrenomyeloneuropathy (AMN) Research Study |
Recent Publications/Presentations:
Mosser J, Douar A-M, Sarde C-O, Kioschis P, Feil R, Moser H, Poustka A-M, Mandel J-L, and Aubourg P, 1993. Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters. Nature 361:726-730.
Moser HW, 1995. Adrenoleukodystrophy: Natural history, treatment and outcome. The Komrower Lecture. J Inher Metab Dis 18:435-447.
Moser HW, 1997. Adrenoleukodystrophy: Phenotype, genetics, pathogenesis, and therapy. Brain 120:1485-1508.
Moser HW, Moser AB, Frayer KK, Chen W, Schulman JD, O'Neill BP, and Kishimoto Y, 1998. Landmark article: Adrenoleukodystrophy: Increased plasma content of saturated very long chain fatty acids. Neurology 51(2):334.
Moser HW, Loes DJ, Melhem ER, Raymond GV, Bezman L, Cox CS, and Lu S-E, 2000. The Peter Emil Becker Memorial Lecture: X-linked adrenoleukodystrophy: Overview and prognosis as a function of age and brain magnetic resonance imaging abnormality. A study involving 372 patients. Neuropediatrics 31:227-239.
Moser HW, 1995. Adrenoleukodystrophy: Natural history, treatment and outcome. The Komrower Lecture. J Inher Metab Dis 18:435-447.
Moser HW, 1997. Adrenoleukodystrophy: Phenotype, genetics, pathogenesis, and therapy. Brain 120:1485-1508.
Moser HW, Moser AB, Frayer KK, Chen W, Schulman JD, O'Neill BP, and Kishimoto Y, 1998. Landmark article: Adrenoleukodystrophy: Increased plasma content of saturated very long chain fatty acids. Neurology 51(2):334.
Moser HW, Loes DJ, Melhem ER, Raymond GV, Bezman L, Cox CS, and Lu S-E, 2000. The Peter Emil Becker Memorial Lecture: X-linked adrenoleukodystrophy: Overview and prognosis as a function of age and brain magnetic resonance imaging abnormality. A study involving 372 patients. Neuropediatrics 31:227-239.
Contact Information:
Hugo W. Moser, M.D.
Director, Neurogenetics Research Center Kennedy Krieger Institute
University Professor of Neurology and Pediatrics
Johns Hopkins University
Kennedy Krieger Institute
707 North Broadway
Baltimore, MD 21205
Telephone and Email contacts
Telephone: (443) 923-2750
Ann Snitcher, Secretary
snitcher@kennedykrieger.org
(443) 923-2766
Kim Hollandsworth
hollandsworth@kennedykrieger.org
(443) 923-2772
Faculty | Clinical Programs | Research
Director, Neurogenetics Research Center Kennedy Krieger Institute
University Professor of Neurology and Pediatrics
Johns Hopkins University
Kennedy Krieger Institute
707 North Broadway
Baltimore, MD 21205
Telephone and Email contacts
Telephone: (443) 923-2750
Ann Snitcher, Secretary
snitcher@kennedykrieger.org
(443) 923-2766
Kim Hollandsworth
hollandsworth@kennedykrieger.org
(443) 923-2772