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Lhermitte-Duclos disease: a report of 31 cases with immunohistochemical analysis of the PTEN/AKT/mTOR pathway.
Abel TW, Baker SJ, Fraser MM, Tihan T, Nelson JS, Yachnis AT, Bouffard JP, Mena H, Burger PC, Eberhart CG
(2005) J Neuropathol Exp Neurol 64: 341-9
MeSH Terms: Adolescent, Adult, Cerebellar Neoplasms, Female, Ganglioneuroma, Hamartoma Syndrome, Multiple, Humans, Immunohistochemistry, Male, Middle Aged, PTEN Phosphohydrolase, Phosphoric Monoester Hydrolases, Protein Kinases, Protein-Serine-Threonine Kinases, Proto-Oncogene Proteins, Proto-Oncogene Proteins c-akt, Signal Transduction, TOR Serine-Threonine Kinases, Thyroid Diseases, Tumor Suppressor Proteins
Show Abstract · Added March 5, 2014
Lhermitte-Duclos disease (LDD) is a rare cerebellar tumor associated with Cowden disease (CD) and germline mutations in the PTEN gene. To further define these relationships, we reviewed clinical and pathologic findings in 31 LDD cases and analyzed the status of the PTEN pathway in 11 of them. We hypothesized that the granule cell hypertrophy in LDD is secondary to activation of mammalian target of rapamycin (mTOR), a downstream effector in the PTEN/AKT pathway and a major regulator of cell growth. Histopathologically, in addition to the classical findings of LDD, we observed prominent vascular proliferation and vacuolization of the white matter in many of the lesions. Four patients met diagnostic criteria for CD, and many of the remaining patients had some clinical features of CD. Immunohistochemical analysis showed high levels of phospho-AKT and phospho-S6 in the large ganglionic cells forming the lesions, indicating activation of the PTEN/AKT/mTOR pathway and suggesting a central role for mTOR in the pathogenesis of LDD. These data support recommendations for genetic testing and screening for CD in patients with LDD and suggest a novel therapy for LDD through pharmacologic inhibition of mTOR.
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20 MeSH Terms
Medulloblastoma growth inhibition by hedgehog pathway blockade.
Berman DM, Karhadkar SS, Hallahan AR, Pritchard JI, Eberhart CG, Watkins DN, Chen JK, Cooper MK, Taipale J, Olson JM, Beachy PA
(2002) Science 297: 1559-61
MeSH Terms: Animals, Antineoplastic Agents, Bicuculline, Cell Differentiation, Cell Division, Cerebellar Neoplasms, Disease Models, Animal, Hedgehog Proteins, Humans, Medulloblastoma, Membrane Proteins, Mice, Mice, Nude, Patched Receptors, Receptors, Cell Surface, Signal Transduction, Trans-Activators, Tumor Cells, Cultured
Show Abstract · Added March 5, 2014
Constitutive Hedgehog (Hh) pathway activity is associated with initiation of neoplasia, but its role in the continued growth of established tumors is unclear. Here, we investigate the therapeutic efficacy of the Hh pathway antagonist cyclopamine in preclinical models of medulloblastoma, the most common malignant brain tumor in children. Cyclopamine treatment of murine medulloblastoma cells blocked proliferation in vitro and induced changes in gene expression consistent with initiation of neuronal differentiation and loss of neuronal stem cell-like character. This compound also caused regression of murine tumor allografts in vivo and induced rapid death of cells from freshly resected human medulloblastomas, but not from other brain tumors, thus establishing a specific role for Hh pathway activity in medulloblastoma growth.
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18 MeSH Terms