Ann B. Moser

Ann B. Moser
Ann Moser
Co-Director, Peroxisomal Diseases Laboratory

Kennedy Krieger Institute
707 N. Broadway
Baltimore, MD 21205
Phone: (443) 923-2761
Email: mosera@kennedykrieger.org

Ann Moser is a research associate in neurology and co-director of the Peroxisomal Diseases Laboratory at Kennedy Krieger Institute and Johns Hopkins University.


Biographical Sketch:

Ann Moser received a bachelor’s degree in biochemistry in 1961 from Radcliffe College. During the time she was an undergraduate, she was a technician in Dr. Konrad Bloch’s laboratory at Harvard University. After working as a technician in laboratories in different hospitals, Moser joined the John F. Kennedy Institute (later Kennedy Krieger Institute) in 1976 as a senior technician. In 1982, she became an assistant in neurology. Since 1991, Moser has been working as a research associate in neurology. She is a co-director of the Peroxisomal Diseases Laboratory in the Hugo W. Moser Research Institute at the Kennedy Krieger Institute.


Research Summary:

The peroxisomal diseases laboratory receives approximately 100 blood samples per week for the analysis of total lipid fatty acids, including the very long chain fatty acids, essential fatty acids and branched chain fatty acids. Individuals with increased plasma, very long chain fatty acids and or branched chain fatty acids have disorders of peroxisomal metabolism. The peroxisomal disorders include patients with X-linked adrenoleukodystrophy, the Zellweger spectrum disorders, rhizomelic chondrodysplasia punctata and adult Refsum’s disease. Under the leadership of Ali Fatemi, we are measuring the plasma and red blood cell fatty acids to follow the dietary therapy of peroxisomal disorders with oils such as Lorenzo’s oil and fish or algae oils (high w3 fatty acid oils). For research collaborators from the Kennedy Krieger Institute, Johns Hopkins and/or medical centers outside of Baltimore, we measure plasma and red blood cell fatty acids in patients with other diseases such as retinitis pigmentosa, heart disease, cystic fibrosis, type 2 diabetes, chronic seizure disorders, Downs syndrome, ADHD, Alzheimer disease, kidney transplant recipients and autism. In addition to our human fatty acid analyses, we provide fatty acid analyses for various transgenic mouse models including the Zellweger mouse, the X-linked adrenoleukodystrophy mouse and the obese mouse. Our current research focus is to develop a neonatal screening test for X-linked adrenoleukodystrophy (ALD) by using the newborn blood spot that is collected on all US babies at birth. In December 2008, together with the MD State Newborn Screening Laboratory, we started a pilot study screening for ALD in 5000 newborns born in the local Baltimore hospitals.

LINK: SciVal Experts Research Profile for Ann Moser


Research Publications: 

Gauthier N., Wu J.W., Wang S.P., Allard P., Mamer O.A., Sweetman L., Moser A.B., Kratz L., Alvarez F., Robitaille Y., Lépine F., Mitchell G.A. (2013). A Liver-Specific Defect of Acyl-CoA Degradation Produces Hyperammonemia, Hypoglycemia and a Distinct Hepatic Acyl-CoA Pattern. PLOS ONE, 8(7), e60581.

Itoyama A., Honsho M., Abe Y., Moser A., Yoshida Y., Fujiki Y. (2012). Docosahexaenoic acid mediates peroxisomal elongation, a prerequisite for peroxisome division. Journal of Cell Science, 125(Pt3), 589-602.

Itzkovitz B., Jiralerspong S., Nimmo G., Loscalzo M., Horovitz D.D., Snowden A., Moser A., Steinberg S., Braverman N. (2012). Functional characterization of novel mutations in GNPAT and AGPS, causing rhizomelic chondrodysplasia punctata (RCDP) types 2 and 3. Human Mutation, 33(1), 189-197.

Engelen M., Tran L., Ofman R., Brennecke J., Moser A.B., Dijkstra I.M., Wanders R.J., Poll-The BT, Kemp S. (2012). Bezafibrate for X-linked adrenoleukodystrophy. PLOS One, 7(7), e41013.

Moser A.B., Steinberg S.J., Watkins P.A., Moser H.W., Ramaswamy K., Siegmund K.D., Lee D.R., Ely J.J., Ryder O.A., Hacia J.G. (2011). Human and great ape red blood cells differ in plasmalogen levels and composition. Lipids in Health & Disease, 10,101.

Watkins P. A., Moser A. B., Toomer C. B., Steinberg S. J., Moser H. W., Karaman M. W., et al. (2010). Identification of differences in human and great ape phytanic acid metabolism that could influence gene expression profiles and physiological functions. BMC Physiology, 10,19.

Hubbard, W. C., Moser A. B., Liu A. C., Jones R. O., Steinberg S. J., Lorey F., et al. (2009). Newborn screening for X-linked adrenoleukodystrophy (X-ALD): validation of a combined liquid chromatography-tandem mass spectrometric (LC-MS/MS) method. Molecular Genetics and Metabolism, 97(3), 212-220.

Hubbard, W. C., Moser A. B., Liu A. C., Jones R. O., Steinberg S. J., Lorey F., et al. (2009). Newborn screening for X-linked adrenoleukodystrophy (X-ALD): validation of a combined liquid chromatography-tandem mass spectrometric (LC-MS/MS) method. Molecular Genetics and Metabolism, 97(3), 212-220.

Savransky V., Jun J., Li J., Nanayakkara A., Fonti S., Moser A. B., et al. (2008). Dyslipidemia and atherosclerosis induced by chronic intermittent hypoxia are attenuated by deficiency of stearoyl coenzyme A desaturase. Circulation Research, 103(10), 1173-1180.

Moser, H. W., Moser A. B., Hollandsworth K., Brereton H. N., & Raymond G. V. (2007). 'Lorenzo's oil' therapy for X-linked adrenoleukodystrophy: rationale and current assessment of efficacy. Journal of Molecular Neuroscience, 33(1), 105-113.

Lu, J. F., Barron-Casella E., Deering R., Heinzer A. K., Moser A. B., deMesy Bentley K. L., et al. (2007). The role of peroxisomal ABC transporters in the mouse adrenal gland: the loss of Abcd2 (ALDR), Not Abcd1 (ALD), causes oxidative damage. Laboratory Investigation, 87(3), 261-272.

Raymond G. V., Jones R. O. and Moser A. B. (2007). Newborn screening for adrenoleukodystrophy: Implications for therapy. Molecular Diagnosis & Therapy, 11(6), 381-384.

Hubbard W. C., Moser A. B., Tortorelli S., Liu A., Jones D. and Moser H.(2007). Combined liquid chromatography-tandem mass spectrometry as an analytical method for high throughput screening for X-linked adrenoleukodystrophy and other peroxisomal disorders: preliminary findings.. Molecular Diagnosis & Metabolism, 89(1-2), 185-187.

Cox C. S., Dubey P., Raymond G. V., Mahmood A., Moser A. B. and Moser H. W.(2006). Cognitive evaluation of neurologically asymptomatic boys with X-linked adrenoleukodystrophy. Archives of Neurology, 63(1), 69-73.

Steinberg S. J., Dodt G., Raymond G. V., Braverman N. E., Moser A. B. and Moser H. W. (2006). Cognitive evaluation of neurologically asymptomatic boys with X-linked adrenoleukodystrophy. Archives of Neurology, 63(1), 69-73.

Moser H. W., Raymond G. V., Lu S. E., Muenz L. R., Moser A. B., Xu J., et al. (2006). Follow-up of 89 asymptomatic patients with adrenoleukodystrophy treated with Lorenzo's oil. Archives of Neurology, 62(7), 1073-1080.

Dubey, P., Raymond G. V., Moser A. B., Kharkar S., Bezman L., & Moser H. W. (2006). Adrenal insufficiency in asymptomatic adrenoleukodystrophy patients identified by very long-chain fatty acid screening. The Journal of Pediatrics, 146(4), 528-532.

Heinzer A. K., Watkins P. A., Lu J. F., Kemp S., Moser A. B., Li Y., et al. (2003). A very long-chain acyl-CoA synthetase-deficient mouse and its relevance to X-linked adrenoleukodystrophy. Human Molecular Genetics, 12(10), 1145-1154.

Li Z., Yang S., Lin H., Huang J., Watkins P. A., Moser A. B., et al. (2003). Probiotics and antibodies to TNF inhibit inflammatory activity and improve nonalcoholic fatty liver disease. Hepatology, 37(2), 343-350.

Tagawa Y., Laroy W., Nimrichter L., Fromholt S. E., Moser A. B., Moser H. W., et al (2002). Anti-ganglioside antibodies bind with enhanced affinity to gangliosides containing very long chain fatty acids. Neurochemical Research, 27(7-8), 847-855.