Genomes and Genes
Richard L Sidman
Affiliation: Harvard University
- Unique glycerophospholipid signature in retinal stem cells correlates with enzymatic functions of diverse long-chain acyl-CoA synthetasesJianxue Li
Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA
Stem Cells 25:2864-73. 2007..These novel findings suggest that LACS-associated GPL signature and cell membrane fluidity may participate in regulating proliferation versus differentiation in RSCs and, perhaps, other types of stem cells...
- Glucosylceramide synthase inhibition alleviates aberrations in synucleinopathy models.S Pablo Sardi
Proc Natl Acad Sci U S A 114:2699-2704. 2017..Hence, inhibition of GCS represents a disease-modifying therapeutic strategy for GBA-related synucleinopathies and conceivably for certain forms of sporadic disease...
- The peptidomimetic Vasotide targets two retinal VEGF receptors and reduces pathological angiogenesis in murine and nonhuman primate models of retinal diseaseRichard L Sidman
Harvard Medical School and Department of Neurology, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA
Sci Transl Med 7:309ra165. 2015..This prototype drug candidate is a promising new dual receptor inhibitor of the VEGF ligand with potential for translation into safer, less-invasive applications to combat pathological angiogenesis in retinal disorders. ..
- Temporal neuropathologic and behavioral phenotype of 6neo/6neo Pompe disease miceRichard L Sidman
Department of Neurology RLS, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA
J Neuropathol Exp Neurol 67:803-18. 2008..A better understanding of the basis for clinical manifestations is needed to correlate CNS pathology with Pompe disease manifestations...
- Injection of mouse and human neural stem cells into neonatal Niemann-Pick A model miceRichard L Sidman
Department of Neurology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02115, USA
Brain Res 1140:195-204. 2007..However, though Purkinje cell rescue has been achieved with NSCs in some other disease models, loss of Purkinje neurons and decline in rotorod performance were still present in injected ASMKO mice...
- Nna1 mediates Purkinje cell dendritic development via lysyl oxidase propeptide and NF-κB signalingJianxue Li
Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, USA
Neuron 68:45-60. 2010..These findings provide insight into Nna1's role in neuronal development and why its absence renders Purkinje cells more vulnerable...
- Tissue plasminogen activator regulates Purkinje neuron development and survivalJianxue Li
Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA
Proc Natl Acad Sci U S A 110:E2410-9. 2013..In sum, excess tPA/plasmin, through separate downstream molecular mechanisms, regulates postnatal PN dendritogenesis, synaptogenesis, mitochondrial structure and function, and selective PN viability. ..
- Neural stem cells rescue nervous purkinje neurons by restoring molecular homeostasis of tissue plasminogen activator and downstream targetsJianxue Li
Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02115, USA
J Neurosci 26:7839-48. 2006..This report demonstrates for the first time that NSCs can rescue imperiled host neurons by rectifying their gene expression, elevating somatic stem cell therapeutic potential beyond solely cell replacement strategy...
- Expression profile of an operationally-defined neural stem cell cloneMark A Parker
Department of Otolaryngology, EN41, Children s Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USA
Exp Neurol 194:320-32. 2005..Furthermore, when operational definitions are employed, a common set of stem-like genes does emerge across both embryonic and somatic stem cells of various organ systems, including the nervous system...
- Physical activity-mediated functional recovery after spinal cord injury: potential roles of neural stem cellsYang D Teng
Department of Neurosurgery, Harvard Medical School, Brigham and Women s Hospital and Children s Hospital Boston, Boston, MA 02115, USA
Regen Med 1:763-76. 2006....
- Purkinje neuron degeneration in nervous (nr) mutant mice is mediated by a metabolic pathway involving excess tissue plasminogen activatorJianxue Li
Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, USA
Proc Natl Acad Sci U S A 103:7847-52. 2006..We thus propose a molecular pathway by which the excessive tPA in nr cerebellum mediates PN degeneration...
- Targeted Treatment of Experimental Spinal Cord Glioma With Dual Gene-Engineered Human Neural Stem CellsAlexander E Ropper
Department of Neurosurgery, Brigham and Women s Hospital, Harvard Medical School, Boston, Massachusetts Division of SCI Research, Veterans Affairs Boston Healthcare System, Boston, Massachusetts Medical Research Institute, Chung Ang University College of Medicine, Seoul, Korea Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts Stem Cell Center, Sanford Burnham Medical Research Institute, La Jolla, California Department of Medicine, University of British Columbia, Vancouver, BC, Canada Department of PM and R, Spaulding Rehabilitation Hospital, Harvard Medical School, Boston, Massachusetts
Neurosurgery 79:481-91. 2016..There are currently no satisfactory treatments or experimental models showing autonomic dysfunction for intramedullary spinal cord gliomas (ISCG)...
- Toll-like receptor 8 functions as a negative regulator of neurite outgrowth and inducer of neuronal apoptosisYinghua Ma
Department of Neurology, Beth Israel Deaconess Medical Center, Boston, MA 02115, USA
J Cell Biol 175:209-15. 2006..These findings reveal novel functions for TLR8 in the mammalian nervous system that are distinct from the classical role of TLRs in immunity...
- Atm-, p53-, and Gadd45a-deficient mice show an increased frequency of homologous recombination at different stages during developmentAlexander J R Bishop
Department of Genetics, Harvard Medical School, Boston, Massachusetts 02115, USA
Cancer Res 63:5335-43. 2003..This temporal resolution suggests that Atm and p53 can act to maintain genomic integrity by different mechanisms in certain in vivo contexts...
- Directed migration of neural stem cells to sites of CNS injury by the stromal cell-derived factor 1alpha/CXC chemokine receptor 4 pathwayJaime Imitola
Center for Neurologic Diseases, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, USA
Proc Natl Acad Sci U S A 101:18117-22. 2004..CXCR4 expression within germinal zones suggests that NSC homing after injury and migration during development may invoke similar mechanisms...
- Multimodal actions of neural stem cells in a mouse model of ALS: a meta-analysisYang D Teng
Departments of Neurosurgery and PM and R, Brigham and Women s Hospital, Spaulding Rehabilitation Hospital and Harvard Medical School, Boston, MA 02115, USA
Sci Transl Med 4:165ra164. 2012..We conclude that the widespread, pleiotropic, modulatory actions exerted by transplanted NSCs may represent an accessible therapeutic application of stem cells for treating ALS and other untreatable degenerative diseases...
- Alleviation of chronic pain following rat spinal cord compression injury with multimodal actions of huperzine ADou Yu
Department of Neurosurgery, Harvard Medical School Brigham and Women s Hospital, Boston, MA 02115, USA
Proc Natl Acad Sci U S A 110:E746-55. 2013..Therefore, HUP-A may provide safe and effective management for chronic postneurotrauma pain by reestablishing homeostasis of sensory circuits...
- TLR8: an innate immune receptor in brain, neurons and axonsYinghua Ma
Department of Neurology, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA
Cell Cycle 6:2859-68. 2007....
- Multimodal coherent anti-Stokes Raman scattering microscopy reveals microglia-associated myelin and axonal dysfunction in multiple sclerosis-like lesions in miceJaime Imitola
Brigham and Women s Hospital, Center for Neurologic Diseases, Partner Multiple Sclerosis Center, Harvard Medical School, Department of Neurology, Boston, Massachusetts 02115, USA
J Biomed Opt 16:021109. 2011....
- Functional multipotency of stem cells: a conceptual review of neurotrophic factor-based evidence and its role in translational researchYang D Teng
Division of SCI Research, Veterans Affairs Boston Healthcare System, Boston, MA, USA
Curr Neuropharmacol 9:574-85. 2011..We suggest that embracing this view of the "multipotency" of the SCs is pivotal for correctly, efficiently, and optimally exploiting stem cell biology for therapeutic applications, including reconstitution of a dysfunctional CNS...
- Stem cells: cross-talk and developmental programsJaime Imitola
Center for Neurologic Diseases, Department of Neurology, Brigham and Women s Hospital, Boston, MA4 02115, USA
Philos Trans R Soc Lond B Biol Sci 359:823-37. 2004..Understanding the relevant molecular pathways of stem cell repair phenotype should be a priority, in our view, for the entire stem cell field...
- Integrin-linked kinase is required for laminin-2-induced oligodendrocyte cell spreading and CNS myelinationSoo Jin Chun
Department of Neurology, Beth Israel Deaconess Medical Center, Center for Neurodegeneration and Repair and the Program in Neuroscience, Harvard Medical School, Boston, MA 02115, USA
J Cell Biol 163:397-408. 2003..A dominant negative ILK inhibits LN-2-induced myelinlike membrane formation. A critical component of the myelination signaling cascade includes LN-2 and integrin signals through ILK...
- A mouse model of classical late-infantile neuronal ceroid lipofuscinosis based on targeted disruption of the CLN2 gene results in a loss of tripeptidyl-peptidase I activity and progressive neurodegenerationDavid E Sleat
Center for Advanced Biotechnology and Medicine, University of Medicine and Dentistry of New Jersey, Piscataway, New Jersey 08854, USA
J Neurosci 24:9117-26. 2004..In addition, the CLN2-targeted mouse also represents a valuable model for the evaluation of different therapeutic strategies...
- Purkinje cell degeneration (pcd) phenotypes caused by mutations in the axotomy-induced gene, Nna1Angeles Fernandez-Gonzalez
Department of Developmental Neurobiology, St Jude Children s Research Hospital, Memphis, TN 38105, USA
Science 295:1904-6. 2002..The present study suggests an unexpected molecular link between neuronal degeneration and regeneration, and its results have potential implications for neurodegenerative diseases and male infertility...
- Intrinsic resistance of neural stem cells to toxic metabolites may make them well suited for cell non-autonomous disorders: evidence from a mouse model of Krabbe leukodystrophyRoseanne M Taylor
Department of Animal Science, Faculty of Veterinary Science, University of Sydney and New South Wales, Australia
J Neurochem 97:1585-99. 2006..Most neurological disease are complex in this way and will likely require multifaceted approaches, perhaps with NSCs serving as the 'glue'...
- Neural stem cells as novel cancer therapeutic vehiclesStephen Yip
Department of Pathology and Laboratory Medicine, Vancouver General Hospital, University of British Columbia, Vancouver, BC, Canada
Eur J Cancer 42:1298-308. 2006..These aspects are reviewed and discussed here...
- Behavioral improvement in a primate Parkinson's model is associated with multiple homeostatic effects of human neural stem cellsD Eugene Redmond
Department of Psychiatry, Yale University School of Medicine, New Haven, CT 06510, USA
Proc Natl Acad Sci U S A 104:12175-80. 2007..We propose that multiple modes of reciprocal interaction between exogenous hNSCs and the pathological host milieu underlie the functional improvement observed in this model of PD...
- Treatment of hypoxia-induced retinopathy with targeted proapoptotic peptidomimetic in a mouse model of diseaseJohanna Lahdenranta
The University of Texas, M D Anderson Cancer Center, Houston, TX 77030, USA
FASEB J 21:3272-8. 2007..Targeted proapoptotic peptides may prove useful in the management of angiogenic retinal diseases...
- Acute injury directs the migration, proliferation, and differentiation of solid organ stem cells: evidence from the effect of hypoxia-ischemia in the CNS on clonal "reporter" neural stem cellsKook In Park
Department of Pediatrics and The Brain Korea 21 Project for Medical Sciences, Yonsei University College of Medicine, Seoul, Korea
Exp Neurol 199:156-78. 2006....
- Aminopeptidase A is a functional target in angiogenic blood vesselsSerena Marchio
The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA
Cancer Cell 5:151-62. 2004..Finally, we successfully treated tumor-bearing mice with APA binding peptides or anti-APA blocking monoclonal antibodies. These data show that APA is a regulator of blood vessel formation, and can serve as a functional vascular target...
- Impaired angiogenesis in aminopeptidase N-null miceRoberto Rangel
Department of Genitourinary Medical Oncology, University of Texas M D Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA
Proc Natl Acad Sci U S A 104:4588-93. 2007..Finally, in addition to vascular biology research, APN-null mice may be useful reagents in other medical fields such as malignant, cardiovascular, immunological, or infectious diseases...