Genomes and Genes
Affiliation: Massachusetts General Hospital
- Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondriaPiera Pasinelli
Day Laboratory for Neuromuscular Research, Department of Neurology, Harvard Medical School, Mass General Institute for Neurodegenerative Disease, Massachusetts General Hospital, Charlestown, MA 02129, USA
Neuron 43:19-30. 2004..These findings provide new insights into the anti-apoptotic function of SOD1 and suggest that entrapment of Bcl-2 by large SOD1 aggregates may deplete motor neurons of this anti-apoptotic protein...
- A caspase-3-cleaved fragment of the glial glutamate transporter EAAT2 is sumoylated and targeted to promyelocytic leukemia nuclear bodies in mutant SOD1-linked amyotrophic lateral sclerosisStuart L Gibb
Farber Institute for Neurosciences, Weinberg Unit for ALS Research, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, and Cecil B Day Laboratory for Neuromuscular Research, Massachusetts General Hospital, Charlestown 02129, USA
J Biol Chem 282:32480-90. 2007....
- Caspase-3 cleaves and inactivates the glutamate transporter EAAT2William Boston-Howes
Department of Neurology, MassGeneral Institute for Neurodegenerative Disease, Harvard Medical School, Charlestown, Massachusetts 02129, USA
J Biol Chem 281:14076-84. 2006..Taken together, our findings suggest that caspase-3 cleavage of EAAT2 is one mechanism responsible for the impairment of glutamate uptake in mutant SOD1-linked ALS...
- Molecular biology of amyotrophic lateral sclerosis: insights from geneticsPiera Pasinelli
Day Neuromuscular Research Laboratory, Massachusetts General Hospital, Room 3125, Building 114, 16th Street, Navy Yard, Charlestown, Massachusetts 02429, USA
Nat Rev Neurosci 7:710-23. 2006..Here, we present an overview of the mechanisms for motor neuron death and of the role of non-neuronal cells in ALS...
- RNA interference-mediated silencing of mutant superoxide dismutase rescues cyclosporin A-induced death in cultured neuroblastoma cellsMichele M Maxwell
Day Laboratory for Neuromuscular Research and High Throughput Screening Laboratory, MassGeneral Institute for Neurodegenerative Disease, Massachusetts General Hospital and Harvard Medical School, Charlestown, MA 02129, USA
Proc Natl Acad Sci U S A 101:3178-83. 2004..The present study further supports the therapeutic potential of RNAi-based methods for the treatment of inherited human diseases, including ALS...
- Inhibition of SOD1 expression by mitomycin C is a non-specific consequence of cellular toxicityWendy J Broom
Day Neuromuscular Research Laboratory, Massachusetts General Hospital, MGH East, 114 16th Street, Navy Yard, Charlestown, MA 02129, USA
Neurosci Lett 393:184-8. 2006..Our data indicate the apparent inhibition of SOD1 expression by MC is a non-specific consequence of MC-induced cellular toxicity...
- Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALSDaryl A Bosco
Department of Neurology, University of Massachusetts Medical Center, Worcester, Massachusetts, USA
Nat Neurosci 13:1396-403. 2010..Our findings suggest that wild-type SOD1 can be pathogenic in SALS and identify an SOD1-dependent pathogenic mechanism common to FALS and SALS...
- Molecular signature of late-stage human ALS revealed by expression profiling of postmortem spinal cord gray matterFernando Dangond
Laboratory of Transcriptional and Immune Regulation, Center for Neurologic Diseases, Brigham and Women's Hospital, Department of Neurology, Harvard Medical School, Boston, Massachusetts 02115, USA
Physiol Genomics 16:229-39. 2004..It is apparent from this study that DNA microarray analysis and appropriate bioinformatics can reveal distinct phenotypic changes that underlie the terminal stages of neurodegeneration in ALS...
- Prophylactic creatine administration mediates neuroprotection in cerebral ischemia in miceShan Zhu
Neuroapoptosis Laboratory, Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
J Neurosci 24:5909-12. 2004..Prophylactic creatine supplementation, similar to what is recommended for an agent such as aspirin, may be considered for patients in high stroke-risk categories...
- Nordihydroguaiaretic acid increases glutamate uptake in vitro and in vivo: therapeutic implications for amyotrophic lateral sclerosisWilliam Boston-Howes
Weinberg Unit for ALS Research, Farber Institute for the Neurosciences, Thomas Jefferson University, Philadelphia, PA 19107, USA
Exp Neurol 213:229-37. 2008....