Vijaya Ramesh

Summary

Affiliation: Harvard University
Country: USA

Publications

  1. ncbi Phosphorylation of tuberin as a novel mechanism for somatic inactivation of the tuberous sclerosis complex proteins in brain lesions
    Sangyeul Han
    Molecular Neurogenetics Unit, Molecular Neuro Oncology, and Department of Neurology, Harvard Medical School Massachusetts General Hospital, Charlestown, Massachusetts, USA
    Cancer Res 64:812-6. 2004
  2. ncbi TorsinB--perinuclear location and association with torsinA
    Jeffrey W Hewett
    Departments of Neurology and Radiology, Molecular Neurogenetics Unit, Massachusetts General Hospital, 13th Street, Charlestown, MA 02129, USA
    J Neurochem 89:1186-94. 2004
  3. ncbi Magicin, a novel cytoskeletal protein associates with the NF2 tumor suppressor merlin and Grb2
    Thorsten Wiederhold
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Charlestown, MA 02129, USA
    Oncogene 23:8815-25. 2004
  4. pmc Lack of association of rare functional variants in TSC1/TSC2 genes with autism spectrum disorder
    Samira Bahl
    Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA
    Mol Autism 4:5. 2013
  5. ncbi Merlin and the ERM proteins in Schwann cells, neurons and growth cones
    Vijaya Ramesh
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Charlestown, Massachusetts 02129, USA
    Nat Rev Neurosci 5:462-70. 2004
  6. ncbi Aspects of tuberous sclerosis complex (TSC) protein function in the brain
    V Ramesh
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Building 149, 13th Street, Charlestown, MA 02129, USA
    Biochem Soc Trans 31:579-83. 2003
  7. ncbi Pam and its ortholog highwire interact with and may negatively regulate the TSC1.TSC2 complex
    Vanishree Murthy
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Charlestown, Massachusetts 02129, USA
    J Biol Chem 279:1351-8. 2004
  8. pmc NF2/merlin is a novel negative regulator of mTOR complex 1, and activation of mTORC1 is associated with meningioma and schwannoma growth
    Marianne F James
    Center for Human Genetic Research, Massachusetts General Hospital, Richard B Simches Research Building, 185 Cambridge St, Boston, MA 02114, USA
    Mol Cell Biol 29:4250-61. 2009
  9. ncbi The NF2 tumor suppressor Merlin and the ERM proteins interact with N-WASP and regulate its actin polymerization function
    Nitasha Manchanda
    Molecular Neurogenetics Unit, Center for Human Genetic Research, Massachusetts General Hospital, Charlestown, Massachusetts 02129, USA
    J Biol Chem 280:12517-22. 2005
  10. ncbi The early onset dystonia protein torsinA interacts with kinesin light chain 1
    Christoph Kamm
    Molecular Neurogenetics Unit, Departments of Neurology and Radiology, Massachusetts General Hospital and Neuroscience Program, Harvard Medical School, Boston, MA 02114, USA
    J Biol Chem 279:19882-92. 2004

Collaborators

Detail Information

Publications32

  1. ncbi Phosphorylation of tuberin as a novel mechanism for somatic inactivation of the tuberous sclerosis complex proteins in brain lesions
    Sangyeul Han
    Molecular Neurogenetics Unit, Molecular Neuro Oncology, and Department of Neurology, Harvard Medical School Massachusetts General Hospital, Charlestown, Massachusetts, USA
    Cancer Res 64:812-6. 2004
    ..These results have widespread implications for understanding the tissue specificity of tumor suppressor gene phenotypes...
  2. ncbi TorsinB--perinuclear location and association with torsinA
    Jeffrey W Hewett
    Departments of Neurology and Radiology, Molecular Neurogenetics Unit, Massachusetts General Hospital, 13th Street, Charlestown, MA 02129, USA
    J Neurochem 89:1186-94. 2004
    ..We conclude that torsinB and torsinA are localized in overlapping cell compartments within the same protein complex, and thus may carry out related functions in vivo...
  3. ncbi Magicin, a novel cytoskeletal protein associates with the NF2 tumor suppressor merlin and Grb2
    Thorsten Wiederhold
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Charlestown, MA 02129, USA
    Oncogene 23:8815-25. 2004
    ..These results support a role for merlin in receptor-mediated signaling at the cell surface, and may have implications in the regulation of cytoskeletal reorganization...
  4. pmc Lack of association of rare functional variants in TSC1/TSC2 genes with autism spectrum disorder
    Samira Bahl
    Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA
    Mol Autism 4:5. 2013
    ....
  5. ncbi Merlin and the ERM proteins in Schwann cells, neurons and growth cones
    Vijaya Ramesh
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Charlestown, Massachusetts 02129, USA
    Nat Rev Neurosci 5:462-70. 2004
  6. ncbi Aspects of tuberous sclerosis complex (TSC) protein function in the brain
    V Ramesh
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Building 149, 13th Street, Charlestown, MA 02129, USA
    Biochem Soc Trans 31:579-83. 2003
    ..All these observations suggest that the tuberin-hamartin complex is likely to have distinct functions in the CNS...
  7. ncbi Pam and its ortholog highwire interact with and may negatively regulate the TSC1.TSC2 complex
    Vanishree Murthy
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Charlestown, Massachusetts 02129, USA
    J Biol Chem 279:1351-8. 2004
    ..Therefore, we hypothesize that Pam, through its interaction with tuberin, could regulate the ubiquitination and proteasomal degradation of the tuberin-hamartin complex particularly in the CNS...
  8. pmc NF2/merlin is a novel negative regulator of mTOR complex 1, and activation of mTORC1 is associated with meningioma and schwannoma growth
    Marianne F James
    Center for Human Genetic Research, Massachusetts General Hospital, Richard B Simches Research Building, 185 Cambridge St, Boston, MA 02114, USA
    Mol Cell Biol 29:4250-61. 2009
    ....
  9. ncbi The NF2 tumor suppressor Merlin and the ERM proteins interact with N-WASP and regulate its actin polymerization function
    Nitasha Manchanda
    Molecular Neurogenetics Unit, Center for Human Genetic Research, Massachusetts General Hospital, Charlestown, Massachusetts 02129, USA
    J Biol Chem 280:12517-22. 2005
    ..This novel function of merlin and the ERMs illustrates a mechanism by which these proteins directly exert their effects on actin reorganization and also provides new insight into N-WASP regulation...
  10. ncbi The early onset dystonia protein torsinA interacts with kinesin light chain 1
    Christoph Kamm
    Molecular Neurogenetics Unit, Departments of Neurology and Radiology, Massachusetts General Hospital and Neuroscience Program, Harvard Medical School, Boston, MA 02114, USA
    J Biol Chem 279:19882-92. 2004
    ..These studies suggest that wild-type torsinA undergoes anterograde transport along microtubules mediated by kinesin and may act as a molecular chaperone regulating kinesin activity and/or cargo binding...
  11. doi Regulation of mTOR complex 2 signaling in neurofibromatosis 2-deficient target cell types
    Marianne F James
    Center for Human Genetic Research, Massachusetts General Hospital, Richard B Simches Research Building, 185 Cambridge Street, Boston, MA 02114, USA
    Mol Cancer Res 10:649-59. 2012
    ..These studies may ultimately aid in the development of suitable therapeutics for NF2-associated tumors...
  12. ncbi Alternative splicing in protein associated with Myc (Pam) influences its binding to c-Myc
    TĂșlio M Santos
    Molecular Neurogenetics Unit and Center for Human Genetic Research, Massachusetts General Hospital, Boston, Massachusetts 02114, USA
    J Neurosci Res 83:222-32. 2006
    ..The presence of Pam in many cellular compartments, its spliced variants, as well as its multiple binding partners, including tuberin, make it a complex, yet intriguing protein in the nervous system...
  13. pmc Modeling NF2 with human arachnoidal and meningioma cell culture systems: NF2 silencing reflects the benign character of tumor growth
    Marianne F James
    Molecular Neurogenetics Unit, Center for Human Genetic Research, Massachusetts General Hospital, Richard B Simches Research Building, 185 Cambridge Street, Boston, MA 02114, USA
    Neurobiol Dis 29:278-92. 2008
    ..Merlin suppression by RNAi in arachnoidal cells replicated merlin-deficient meningioma features, thus establishing these cell systems as disease-relevant models for studying NF2 tumorigenesis...
  14. pmc Pam (Protein associated with Myc) functions as an E3 ubiquitin ligase and regulates TSC/mTOR signaling
    Sangyeul Han
    Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA
    Cell Signal 20:1084-91. 2008
    ....
  15. ncbi Magicin associates with the Src-family kinases and is phosphorylated upon CD3 stimulation
    Ming Fen Lee
    Molecular Neurogenetics Unit, Center for Human Genetic Research, Massachusetts General Hospital, 185 Cambridge Street, Boston, MA 02114, USA
    Biochem Biophys Res Commun 348:826-31. 2006
    ....
  16. pmc The E3 ubiquitin ligase protein associated with Myc (Pam) regulates mammalian/mechanistic target of rapamycin complex 1 (mTORC1) signaling in vivo through N- and C-terminal domains
    Sangyeul Han
    Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA
    J Biol Chem 287:30063-72. 2012
    ..The ubiquitin ligase complex containing Pam-Fbxo45 likely targets additional synaptic and axonal proteins, which may explain the overlapping neurodevelopmental defects observed in Phr1 and Fbxo45 deficiency...
  17. ncbi Mediator subunit MED28 (Magicin) is a repressor of smooth muscle cell differentiation
    Kim S Beyer
    Molecular Neurogenetics Unit, Center for Human Genetic Research, Massachusetts General Hospital, Boston 02114, and Department of Cell Biology, Harvard Medical School, Charlestown, Massachusetts 02129, USA
    J Biol Chem 282:32152-7. 2007
    ....
  18. ncbi Distribution and ultrastructural localization of torsinA immunoreactivity in the human brain
    Sarah J Augood
    Neurology Service, Massachusetts General Hospital and Harvard Medical School, CNY 114 2300, 114 16th Street, Charlestown, MA 02129, USA
    Brain Res 986:12-21. 2003
    ....
  19. ncbi Functions of MUC16 in corneal epithelial cells
    Timothy D Blalock
    Department of Ophthalmology, Schepens Eye Research Institute, Harvard Medical School, Boston, Massachusetts 02114, USA
    Invest Ophthalmol Vis Sci 48:4509-18. 2007
    ..The MUC16 cytoplasmic tail has not been characterized, but, because it contains a polybasic amino acid sequence, it potentially interacts with the actin cytoskeleton through ezrin/radixin/moesin (ERM) actin-binding proteins...
  20. pmc Aberrant hyperactivation of akt and Mammalian target of rapamycin complex 1 signaling in sporadic chordomas
    Sangyeul Han
    Center for Human Genetic Research, Massachusetts General Hospital, Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA
    Clin Cancer Res 15:1940-6. 2009
    ..In this study, we investigated whether aberrant TSC/mammalian target of rapamycin complex 1 (mTORC1) signaling pathway is associated with sporadic chordomas...
  21. ncbi A NHERF binding site links the betaPDGFR to the cytoskeleton and regulates cell spreading and migration
    Marianne F James
    Molecular Neurogenetics Unit, Center for Human Genetic Research, Massachusetts General Hospital, Building 149, 13th Street, Charlestown, MA 02129, USA
    J Cell Sci 117:2951-61. 2004
    ..Thus, the interaction of betaPDGFR with NHERF may provide an essential link between the cell membrane and the cortical actin cytoskeleton independent of receptor activity...
  22. ncbi Inactivation patterns of NF2 and DAL-1/4.1B (EPB41L3) in sporadic meningioma
    Fabio Nunes
    Department of Neurology, Massachusetts General Hospital, Building 149, 13th Street, Charlestown, MA 02129, USA
    Cancer Genet Cytogenet 162:135-9. 2005
    ..Furthermore, we found the majority of meningiomas developed monosomy rather than isodisomy at the NF2 and DAL-1/4.1B loci as the mechanism for LOH...
  23. ncbi The TSC1 tumor suppressor hamartin interacts with neurofilament-L and possibly functions as a novel integrator of the neuronal cytoskeleton
    Luciana A Haddad
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Charlestown 02129, USA
    J Biol Chem 277:44180-6. 2002
    ....
  24. ncbi Role of NHERF1, cystic fibrosis transmembrane conductance regulator, and cAMP in the regulation of aquaporin 9
    Christine Pietrement
    Center for Systems Biology, Program in Membrane Biology Nephrology Division, Simches Research Center, Massachusetts General Hospital, 185 Cambridge Street, Boston, MA 02114, USA
    J Biol Chem 283:2986-96. 2008
    ..We propose that CFTR is an important regulator of AQP9 and that the interaction between AQP9, NHERF1, and CFTR may facilitate the activation of AQP9 by cAMP...
  25. ncbi TorsinA in PC12 cells: localization in the endoplasmic reticulum and response to stress
    Jeffrey Hewett
    Molecular Neurogenetics Unit, Department of Neurology, Massachusetts General Hospital, and Neuroscience Program, Harvard Medical School, Boston, Massachusetts, USA
    J Neurosci Res 72:158-68. 2003
    ..Mutant torsinA may interfere with and/or compromise ER functions, especially in dopaminergic neurons, which have high levels of torsinA and are intrinsically vulnerable to oxidative stress...
  26. ncbi A 34 bp deletion within TSC2 is a rare polymorphism, not a pathogenic mutation
    Penelope S Roberts
    Hematology Division, Department of Medicine, Brigham and Women s Hospital, Harvard Medical School, Boston, MA 02115, USA
    Ann Hum Genet 67:495-503. 2003
    ..We also excluded the possibility of mosaicism in the parents with this variant. We conclude that this deletion is a rare polymorphism that does not cause TSC, but may be a modifier of the TSC phenotype...
  27. pmc Genomic profiling distinguishes familial multiple and sporadic multiple meningiomas
    Yiping Shen
    Molecular Neurogenetics Unit, Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA, 02114, USA
    BMC Med Genomics 2:42. 2009
    ....
  28. ncbi TorsinA protein and neuropathology in early onset generalized dystonia with GAG deletion
    Kevin Rostasy
    Pediatric Neurology Floating Hospital, Boston, MA, USA
    Neurobiol Dis 12:11-24. 2003
    ....
  29. pmc Admixture mapping of an allele affecting interleukin 6 soluble receptor and interleukin 6 levels
    David Reich
    Department of Genetics, Harvard Medical School, New Research Building, Boston, MA 02115, USA
    Am J Hum Genet 80:716-26. 2007
    ..0x10-12 for IL-6 SR, and P<2.0x10-9 for IL-6. These results also serve as an important proof of principle, showing that admixture mapping can not only coarsely localize but can also fine map a phenotypically important variant...
  30. ncbi Sacrococcygeal chordomas in patients with tuberous sclerosis complex show somatic loss of TSC1 or TSC2
    Lisa Lee-Jones
    Tumour Molecular Genetics Group, Institute of Medical Genetics, University of Wales College of Medicine, Cardiff, UK
    Genes Chromosomes Cancer 41:80-5. 2004
    ..These data provide the first evidence of a pathogenic role by TSC genes in sacrococcygeal chordomas...
  31. ncbi Layilin, a cell surface hyaluronan receptor, interacts with merlin and radixin
    Petri Bono
    Howard Hughes Medical Institute, Center for Cancer Research, Massachusetts Institute of Technology, Cambridge, MA 02139, USA
    Exp Cell Res 308:177-87. 2005
    ....
  32. pmc Reversal of learning deficits in a Tsc2+/- mouse model of tuberous sclerosis
    Dan Ehninger
    Department of Neurobiology, Brain Research Institute, University of California, Los Angeles, 695 Charles E Young Drive South, Los Angeles, California 90095, USA
    Nat Med 14:843-8. 2008
    ....

Research Grants8

  1. CHARACTERIZATION OF TSC PROTEIN HAMARTIN AND TUBERIN
    Vijaya Ramesh; Fiscal Year: 2005
    ..The information obtained here will elucidate the physiological functions of these tumor suppressors, which will aid in designing better therapies. ..
  2. 2006 NF Consortium for NF1, NF2 and Schwannomatosis
    Vijaya Ramesh; Fiscal Year: 2006
    ..This will promote to the research community at large the status of NF research, the link with other cancers and neurological disorders and the key barriers to be addressed. ..
  3. Genes that deregulate mTOR signaling as candidates for autism spectrum disorders
    Vijaya Ramesh; Fiscal Year: 2007
    ..This project will test whether genes that control mTOR signaling are associated with an increased risk for Autism Spectrum Disorders, and thus has direct relevance to public health. ..