- Natural history of Canavan disease revealed by proton magnetic resonance spectroscopy (1H-MRS) and diffusion-weighted MRI
C G Janson
UMDNJ Robert Wood Johnson Medical School, Dept of Neurosurgery, Camden, NJ 08103, USA
Neuropediatrics 37:209-21. 2006
..The resultant model can be used to non-invasively monitor the natural history of Canavan disease or related leukodystrophies in future studies involving drug, gene therapy, or stem cell treatments...
- Essay: White daisies on my mind (Requiem for an Alzheimer patient)
C G Janson
UMDNJ Robert Wood Johnson Medical School, Camden, NJ 08103, USA
Neurology 65:654-6. 2005
- Immortalization and functional characterization of rat arachnoid cell lines
Department of Neurosurgery, University of Minnesota, Minneapolis, MN 55455, USA
Neuroscience 177:23-34. 2011
- Bowstringing as a complication of deep brain stimulation: case report
Department of Neurosurgery, University of Minnesota, Minneapolis, Minnesota 55455, USA
Neurosurgery 66:E1205; discussion E1205. 2010
..There are no previous reports of this specific complication, which presumably has been more broadly classified under hardware-related complications...
- Mild-onset presentation of Canavan's disease associated with novel G212A point mutation in aspartoacylase gene
Christopher G Janson
Department of Neurology, University of Medicine and Dentistry of New Jersey Robert Wood Johnson Medical School, Camden, NJ, USA
Ann Neurol 59:428-31. 2006
..Future studies of ASPA enzyme structure and gene regulation in these subjects could lead to a better understanding of Canavan's pathophysiology and improvements in ASPA gene therapy...
- Lithium citrate reduces excessive intra-cerebral N-acetyl aspartate in Canavan disease
Robert Wood Johnson Medical School, Neurology, 3 Cooper Plaza, Suite 320, Camden, NJ 08103, USA
Eur J Paediatr Neurol 14:354-9. 2010
..Diffusion Tensor Imaging was available in two patients and suggested micro-structural improvement in the corpus callosum. The results suggest that lithium administration may be beneficial in patients with Canavan disease...
- Serial 1H-MRS in GM2 gangliosidoses
Division of Neurology, University of Medicine and Dentistry of New Jersey, 3 Cooper Plaza, Suite 320, Camden, NJ 08103, USA
Eur J Pediatr 167:347-52. 2008
..1H-MRS may be a valuable tool in monitoring the disease progress and response to therapy in GM2 gangliosidoses. Elevation in the mI may prove to be more sensitive than the other metabolite alterations...
- Effects of AAV-2-mediated aspartoacylase gene transfer in the tremor rat model of Canavan disease
S W J McPhee
Cell and Gene Therapy Center, UMDNJ Robert Wood Johnson Medical School, 401 Haddon Avenue, Suite 395, Camden, NJ 08103, USA
Brain Res Mol Brain Res 135:112-21. 2005
..This study provides evidence that AAV-2-mediated aspartoacylase gene transfer to the brain improves biochemical and behavioral deficits in tremor rat mutants (tm/tm) and supports the rationale of human gene transfer for Canavan disease...
- Global CNS gene transfer for a childhood neurogenetic enzyme deficiency: Canavan disease
CNS Gene Therapy Center, Department of Neurosurgery, Thomas Jefferson University, Philadelphia, PA 19107, USA
Curr Opin Mol Ther 1:487-92. 1999
- Immune responses to AAV in a phase I study for Canavan disease
S W J McPhee
Department of Surgery, Division of Neurosurgery, Robert Wood Johnson Medical School, University of Medicine and Dentistry of New Jersey, 401 Haddon Avenue, Camden, NJ 08103, USA
J Gene Med 8:577-88. 2006
..rAAV-ASPA has been developed for gene delivery to the central nervous system (CNS) for Canavan disease. This study represents the first use of a viral vector in an attempt to ameliorate a neurodegenerative disorder...