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profileVolney L Sheen, Ph.D., M.D.

TitleAssociate Professor of Neurology
InstitutionBeth Israel Deaconess Medical Center
DepartmentNeurology
AddressBeth Israel Deaconess Med Ctr
Cls 641
77 Avenue Louis Pasteur
Boston MA 02115
Phone617/667-0813
Fax617/667-7919
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Collapse research activities and funding
The research activities and funding listed below are automatically derived from NIH ExPORTER and other sources, which might result in incorrect or missing items. Faculty can login to make corrections and additions.
R01NS092062     (SHEEN, VOLNEY L)Feb 1, 2015 - Jan 31, 2020
NIH/NINDS
Apical Abscission and Periventricular Heterotopia: A common basis for disease
Role: Co-Principal Investigator

R01NS063997     (SHEEN, VOLNEY L)Jun 1, 2009 - May 31, 2014
NIH/NINDS
Cellular and Molecular Mechanisms in Periventricular Heterotopia
Role: Principal Investigator

R21HD054347     (SHEEN, VOLNEY L)Apr 4, 2008 - Mar 31, 2011
NIH/NICHD
Human Neural Precursors from CNS Developmental Disorders: Down Syndrome
Role: Principal Investigator

K08MH063886     (SHEEN, VOLNEY L)Jul 16, 2001 - Jun 30, 2007
NIH/NIMH
The Role of Filamin in Periventricular Heterotopias
Role: Principal Investigator

F30MH011034     (SHEEN, VOLNEY L)Nov 26, 1995
NIH/NIMH
LOCAL MICROENVIRONMENTAL CONTROL OF NEURAL SPECIFICATION
Role: Principal Investigator

Collapse Bibliographic 
Collapse selected publications
Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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  1. Hu J, Lu J, Goyal A, Wong T, Lian G, Zhang J, Hecht JL, Feng Y, Sheen VL. Opposing FlnA and FlnB interactions regulate RhoA activation in guiding dynamic actin stress fiber formation and cell spreading. Hum Mol Genet. 2017 Apr 01; 26(7):1294-1304. PMID: 28175289.
    View in: PubMed
  2. Lian G, Dettenhofer M, Lu J, Downing M, Chenn A, Wong T, Sheen V. Filamin A- and formin 2-dependent endocytosis regulates proliferation via the canonical Wnt pathway. Development. 2016 Dec 01; 143(23):4509-4520. PMID: 27789627.
    View in: PubMed
  3. Lu J, Mccarter M, Lian G, Esposito G, Capoccia E, Delli-Bovi LC, Hecht J, Sheen V. Global hypermethylation in fetal cortex of Down syndrome due to DNMT3L overexpression. Hum Mol Genet. 2016 May 01; 25(9):1714-27. PMID: 26911678; PMCID: PMC4986328 [Available on 05/01/17].
  4. Lian G, Sheen VL. Cytoskeletal proteins in cortical development and disease: actin associated proteins in periventricular heterotopia. Front Cell Neurosci. 2015; 9:99. PMID: 25883548; PMCID: PMC4381626.
  5. Liu W, Zhou H, Liu L, Zhao C, Deng Y, Chen L, Wu L, Mandrycky N, McNabb CT, Peng Y, Fuchs PN, Lu J, Sheen V, Qiu M, Mao M, Lu QR. Disruption of neurogenesis and cortical development in transgenic mice misexpressing Olig2, a gene in the Down syndrome critical region. Neurobiol Dis. 2015 May; 77:106-16. PMID: 25747816; PMCID: PMC4428323.
  6. Sheen VL. Filamin A mediated Big2 dependent endocytosis: From apical abscission to periventricular heterotopia. Tissue Barriers. 2014; 2:e29431. PMID: 25097827; PMCID: PMC4117685.
  7. Hu J, Lu J, Lian G, Ferland RJ, Dettenhofer M, Sheen VL. Formin 1 and filamin B physically interact to coordinate chondrocyte proliferation and differentiation in the growth plate. Hum Mol Genet. 2014 Sep 01; 23(17):4663-73. PMID: 24760772; PMCID: PMC4119417.
  8. Sheen VL. Filamin A and Big2: a shared endocytic pathway. Bioarchitecture. 2014 Mar-Apr; 4(2):53-7. PMID: 24709996; PMCID: PMC4199812.
  9. Hu J, Lu J, Lian G, Zhang J, Hecht JL, Sheen VL. Filamin B regulates chondrocyte proliferation and differentiation through Cdk1 signaling. PLoS One. 2014; 9(2):e89352. PMID: 24551245; PMCID: PMC3925234.
  10. Zhang J, Neal J, Lian G, Hu J, Lu J, Sheen V. Filamin A regulates neuronal migration through brefeldin A-inhibited guanine exchange factor 2-dependent Arf1 activation. J Neurosci. 2013 Oct 02; 33(40):15735-46. PMID: 24089482; PMCID: PMC3787497.
  11. Sheen V, Valencia IM, Torres AR. Atypical features in MECP2 P152R-associated Rett syndrome. Pediatr Neurol. 2013 Aug; 49(2):124-6. PMID: 23859859.
    View in: PubMed
  12. Sheen VL, Shankar M, Marin-Valencia I, Bridgemohan CH, Torres AR. Methylphenidate and continuous spike and wave during sleep in a child with attention deficit hyperactivity disorder. Pediatr Neurol. 2013 Jul; 49(1):54-7. PMID: 23827428.
    View in: PubMed
  13. Sheen VL. Periventricular Heterotopia: Shuttling of Proteins through Vesicles and Actin in Cortical Development and Disease. Scientifica (Cairo). 2012; 2012:480129. PMID: 24278701; PMCID: PMC3820590.
  14. Zhang J, Neal J, Lian G, Shi B, Ferland RJ, Sheen V. Brefeldin A-inhibited guanine exchange factor 2 regulates filamin A phosphorylation and neuronal migration. J Neurosci. 2012 Sep 05; 32(36):12619-29. PMID: 22956851; PMCID: PMC3478955.
  15. Tarula E, Ramkissoon S, Pittock S, Sheen VL. Progressive mental status changes and seizures with fluid attenuated inversion recovery (FLAIR) hyperintensity on brain MRI. J Clin Neurosci. 2012 Jun; 19(6):873, 924. PMID: 22764365; PMCID: PMC3613846.
  16. Lian G, Lu J, Hu J, Zhang J, Cross SH, Ferland RJ, Sheen VL. Filamin a regulates neural progenitor proliferation and cortical size through Wee1-dependent Cdk1 phosphorylation. J Neurosci. 2012 May 30; 32(22):7672-84. PMID: 22649246; PMCID: PMC3368379.
  17. Cai L, Lu J, Sheen V, Wang S. Optimal poly(L-lysine) grafting density in hydrogels for promoting neural progenitor cell functions. Biomacromolecules. 2012 May 14; 13(5):1663-74. PMID: 22533450; PMCID: PMC3547621.
  18. Lu J, Lian G, Zhou H, Esposito G, Steardo L, Delli-Bovi LC, Hecht JL, Lu QR, Sheen V. OLIG2 over-expression impairs proliferation of human Down syndrome neural progenitors. Hum Mol Genet. 2012 May 15; 21(10):2330-40. PMID: 22343408; PMCID: PMC3335315.
  19. Cai L, Lu J, Sheen V, Wang S. Promoting nerve cell functions on hydrogels grafted with poly(L-lysine). Biomacromolecules. 2012 Feb 13; 13(2):342-9. PMID: 22251248; PMCID: PMC3538025.
  20. Cai L, Lu J, Sheen V, Wang S. Lubricated biodegradable polymer networks for regulating nerve cell behavior and fabricating nerve conduits with a compositional gradient. Biomacromolecules. 2012 Feb 13; 13(2):358-68. PMID: 22206477; PMCID: PMC3544368.
  21. Sheen VL. Seizure or syncope: lessons over time. J Clin Neurosci. 2012 Mar; 19(3):481-3. PMID: 22245277; PMCID: PMC3278523.
  22. Lu J, Esposito G, Scuderi C, Steardo L, Delli-Bovi LC, Hecht JL, Dickinson BC, Chang CJ, Mori T, Sheen V. S100B and APP promote a gliocentric shift and impaired neurogenesis in Down syndrome neural progenitors. PLoS One. 2011; 6(7):e22126. PMID: 21779383; PMCID: PMC3133657.
  23. Lu J, Delli-Bovi LC, Hecht J, Folkerth R, Sheen VL. Generation of neural stem cells from discarded human fetal cortical tissue. J Vis Exp. 2011 May 25; (51). PMID: 21654623; PMCID: PMC3197109.
  24. Sheen VL, Torres AR, Du X, Barry B, Walsh CA, Kimonis VE. Mutation in PQBP1 is associated with periventricular heterotopia. Am J Med Genet A. 2010 Nov; 152A(11):2888-90. PMID: 20886605; PMCID: PMC3548238.
  25. Sheen VL, Ohaegbulam C, Rencus T, Tandon D. Gabapentin-induced exacerbation of myasthenia gravis. Muscle Nerve. 2010 Jul; 42(1):149. PMID: 20583123; PMCID: PMC3539726.
  26. Ekstein D, Shih LC, Sheen V. Neurological picture. Focal botulinum toxin injections lead to rapid resolution of myogenic artefact during EEG monitoring. J Neurol Neurosurg Psychiatry. 2010 Jun; 81(6):637-8. PMID: 20522871; PMCID: PMC3539720.
  27. Ferland RJ, Batiz LF, Neal J, Lian G, Bundock E, Lu J, Hsiao YC, Diamond R, Mei D, Banham AH, Brown PJ, Vanderburg CR, Joseph J, Hecht JL, Folkerth R, Guerrini R, Walsh CA, Rodriguez EM, Sheen VL. Disruption of neural progenitors along the ventricular and subventricular zones in periventricular heterotopia. Hum Mol Genet. 2009 Feb 01; 18(3):497-516. PMID: 18996916; PMCID: PMC2722192.
  28. Esposito G, Scuderi C, Lu J, Savani C, De Filippis D, Iuvone T, Steardo L, Sheen V, Steardo L. S100B induces tau protein hyperphosphorylation via Dickopff-1 up-regulation and disrupts the Wnt pathway in human neural stem cells. J Cell Mol Med. 2008 Jun; 12(3):914-27. PMID: 18494933; PMCID: PMC3538024.
  29. Pang T, Atefy R, Sheen V. Malformations of cortical development. Neurologist. 2008 May; 14(3):181-91. PMID: 18469675; PMCID: PMC3547618.
  30. Esposito G, Imitola J, Lu J, De Filippis D, Scuderi C, Ganesh VS, Folkerth R, Hecht J, Shin S, Iuvone T, Chesnut J, Steardo L, Sheen V. Genomic and functional profiling of human Down syndrome neural progenitors implicates S100B and aquaporin 4 in cell injury. Hum Mol Genet. 2008 Feb 01; 17(3):440-57. PMID: 17984171.
    View in: PubMed
  31. Lu J, Lian G, Lenkinski R, De Grand A, Vaid RR, Bryce T, Stasenko M, Boskey A, Walsh C, Sheen V. Filamin B mutations cause chondrocyte defects in skeletal development. Hum Mol Genet. 2007 Jul 15; 16(14):1661-75. PMID: 17510210.
    View in: PubMed
  32. Neal J, Takahashi M, Silva M, Tiao G, Walsh CA, Sheen VL. Insights into the gyrification of developing ferret brain by magnetic resonance imaging. J Anat. 2007 Jan; 210(1):66-77. PMID: 17229284; PMCID: PMC2100265.
  33. Lian G, Sheen V. Cerebral developmental disorders. Curr Opin Pediatr. 2006 Dec; 18(6):614-20. PMID: 17099359.
    View in: PubMed
  34. Neal J, Raju GP, Bodell A, Apse K, Walsh CA, Sheen VL. Periventricular heterotopia with complete agenesis of the corpus callosum : a case report. J Neurol. 2006 Oct; 253(10):1358-9. PMID: 16906346.
    View in: PubMed
  35. Neal J, Apse K, Sahin M, Walsh CA, Sheen VL. Deletion of chromosome 1p36 is associated with periventricular nodular heterotopia. Am J Med Genet A. 2006 Aug 01; 140(15):1692-5. PMID: 16835933.
    View in: PubMed
  36. Sheen VL, Ferland RJ, Harney M, Hill RS, Neal J, Banham AH, Brown P, Chenn A, Corbo J, Hecht J, Folkerth R, Walsh CA. Impaired proliferation and migration in human Miller-Dieker neural precursors. Ann Neurol. 2006 Jul; 60(1):137-44. PMID: 16642511.
    View in: PubMed
  37. Ferland RJ, Gaitanis JN, Apse K, Tantravahi U, Walsh CA, Sheen VL. Periventricular nodular heterotopia and Williams syndrome. Am J Med Genet A. 2006 Jun 15; 140(12):1305-11. PMID: 16691586.
    View in: PubMed
  38. Gérard-Blanluet M, Sheen V, Machinis K, Neal J, Apse K, Danan C, Sinico M, Brugières P, Mage K, Ratsimbazafy L, Elbez A, Janaud JC, Amselem S, Walsh C, Encha-Razavi F. Bilateral periventricular heterotopias in an X-linked dominant transmission in a family with two affected males. Am J Med Genet A. 2006 May 15; 140(10):1041-6. PMID: 16596669.
    View in: PubMed
  39. Tsao JW, Neal J, Apse K, Stephan MJ, Dobyns WB, Hill RS, Walsh CA, Sheen VL. Cerebellar ataxia with progressive improvement. Arch Neurol. 2006 Apr; 63(4):594-7. PMID: 16606775.
    View in: PubMed
  40. Sheen VL, Ferland RJ, Neal J, Harney M, Hill RS, Banham A, Brown P, Chenn A, Corbo J, Hecht J, Folkerth R, Walsh CA. Neocortical neuronal arrangement in Miller Dieker syndrome. Acta Neuropathol. 2006 May; 111(5):489-96. PMID: 16456669.
    View in: PubMed
  41. Lu J, Tiao G, Folkerth R, Hecht J, Walsh C, Sheen V. Overlapping expression of ARFGEF2 and Filamin A in the neuroependymal lining of the lateral ventricles: insights into the cause of periventricular heterotopia. J Comp Neurol. 2006 Jan 20; 494(3):476-84. PMID: 16320251.
    View in: PubMed
  42. Masruha MR, Caboclo LO, Carrete H, Cendes IL, Rodrigues MG, Garzon E, Yacubian EM, Sakamoto AC, Sheen V, Harney M, Neal J, Hill RS, Bodell A, Walsh C, Vilanova LC. Mutation in filamin A causes periventricular heterotopia, developmental regression, and West syndrome in males. Epilepsia. 2006 Jan; 47(1):211-4. PMID: 16417552.
    View in: PubMed
  43. Sheen VL, Walsh CA. Periventricular heterotopia: new insights into Ehlers-Danlos syndrome. Clin Med Res. 2005 Nov; 3(4):229-33. PMID: 16303888; PMCID: PMC1288408.
  44. Lu J, Sheen V. Periventricular heterotopia. Epilepsy Behav. 2005 Sep; 7(2):143-9. PMID: 15996530.
    View in: PubMed
  45. Ning MM, Smirnakis S, Furie KL, Sheen VL. Adult acute disseminated encephalomyelitis associated with poststreptococcal infection. J Clin Neurosci. 2005 Apr; 12(3):298-300. PMID: 15851086.
    View in: PubMed
  46. Chang BS, Ly J, Appignani B, Bodell A, Apse KA, Ravenscroft RS, Sheen VL, Doherty MJ, Hackney DB, O'Connor M, Galaburda AM, Walsh CA. Reading impairment in the neuronal migration disorder of periventricular nodular heterotopia. Neurology. 2005 Mar 08; 64(5):799-803. PMID: 15753412.
    View in: PubMed
  47. Sheen VL, Jansen A, Chen MH, Parrini E, Morgan T, Ravenscroft R, Ganesh V, Underwood T, Wiley J, Leventer R, Vaid RR, Ruiz DE, Hutchins GM, Menasha J, Willner J, Geng Y, Gripp KW, Nicholson L, Berry-Kravis E, Bodell A, Apse K, Hill RS, Dubeau F, Andermann F, Barkovich J, Andermann E, Shugart YY, Thomas P, Viri M, Veggiotti P, Robertson S, Guerrini R, Walsh CA. Filamin A mutations cause periventricular heterotopia with Ehlers-Danlos syndrome. Neurology. 2005 Jan 25; 64(2):254-62. PMID: 15668422.
    View in: PubMed
  48. Sheen VL, Basel-Vanagaite L, Goodman JR, Scheffer IE, Bodell A, Ganesh VS, Ravenscroft R, Hill RS, Cherry TJ, Shugart YY, Barkovich J, Straussberg R, Walsh CA. Etiological heterogeneity of familial periventricular heterotopia and hydrocephalus. Brain Dev. 2004 Aug; 26(5):326-34. PMID: 15165674.
    View in: PubMed
  49. Sheen VL, Ganesh VS, Topcu M, Sebire G, Bodell A, Hill RS, Grant PE, Shugart YY, Imitola J, Khoury SJ, Guerrini R, Walsh CA. Mutations in ARFGEF2 implicate vesicle trafficking in neural progenitor proliferation and migration in the human cerebral cortex. Nat Genet. 2004 Jan; 36(1):69-76. PMID: 14647276.
    View in: PubMed
  50. Atri A, Sheen V. Cavernous sinus syndrome and headache due to bilateral carotid artery aneurysms. Arch Neurol. 2003 Sep; 60(9):1327-8. PMID: 12975304.
    View in: PubMed
  51. Sheen VL, Walsh CA. Developmental genetic malformations of the cerebral cortex. Curr Neurol Neurosci Rep. 2003 Sep; 3(5):433-41. PMID: 12914687.
    View in: PubMed
  52. Sheen VL, Topçu M, Berkovic S, Yalnizoglu D, Blatt I, Bodell A, Hill RS, Ganesh VS, Cherry TJ, Shugart YY, Walsh CA. Autosomal recessive form of periventricular heterotopia. Neurology. 2003 Apr 08; 60(7):1108-12. PMID: 12682315.
    View in: PubMed
  53. Sheen VL, Wheless JW, Bodell A, Braverman E, Cotter PD, Rauen KA, Glenn O, Weisiger K, Packman S, Walsh CA, Sherr EH. Periventricular heterotopia associated with chromosome 5p anomalies. Neurology. 2003 Mar 25; 60(6):1033-6. PMID: 12654978.
    View in: PubMed
  54. Sheen VL, Asimakopoulos F, Heyman E, Henderson G, Feske SK. Hemichorea as a presentation of recurrent non-Hodgkin's lymphoma. J Neurol. 2002 Dec; 249(12):1746-8. PMID: 12529805.
    View in: PubMed
  55. Sheen VL, Feng Y, Graham D, Takafuta T, Shapiro SS, Walsh CA. Filamin A and Filamin B are co-expressed within neurons during periods of neuronal migration and can physically interact. Hum Mol Genet. 2002 Nov 01; 11(23):2845-54. PMID: 12393796.
    View in: PubMed
  56. Smith M, Cros D, Sheen V. Hyperperfusion with vasogenic leakage by fMRI in migraine with prolonged aura. Neurology. 2002 Apr 23; 58(8):1308-10. PMID: 11971111.
    View in: PubMed
  57. Nogueira RG, Sheen VL. Images in clinical medicine. Herpes zoster ophthalmicus followed by contralateral hemiparesis. N Engl J Med. 2002 Apr 11; 346(15):1127. PMID: 11948272.
    View in: PubMed
  58. Sheen VL, Dixon PH, Fox JW, Hong SE, Kinton L, Sisodiya SM, Duncan JS, Dubeau F, Scheffer IE, Schachter SC, Wilner A, Henchy R, Crino P, Kamuro K, DiMario F, Berg M, Kuzniecky R, Cole AJ, Bromfield E, Biber M, Schomer D, Wheless J, Silver K, Mochida GH, Berkovic SF, Andermann F, Andermann E, Dobyns WB, Wood NW, Walsh CA. Mutations in the X-linked filamin 1 gene cause periventricular nodular heterotopia in males as well as in females. Hum Mol Genet. 2001 Aug 15; 10(17):1775-83. PMID: 11532987.
    View in: PubMed
  59. Do D, Sheen VL, Bromfield E. Treatment of paroxysmal sympathetic storm with labetalol. J Neurol Neurosurg Psychiatry. 2000 Dec; 69(6):832-3. PMID: 11185641; PMCID: PMC1737162.
  60. Catapano LA, Sheen VL, Leavitt BR, Macklis JD. Differentiation of transplanted neural precursors varies regionally in adults striatum. Neuroreport. 1999 Dec 16; 10(18):3971-7. PMID: 10716243.
    View in: PubMed
  61. Sheen VL, Arnold MW, Wang Y, Macklis JD. Neural precursor differentiation following transplantation into neocortex is dependent on intrinsic developmental state and receptor competence. Exp Neurol. 1999 Jul; 158(1):47-62. PMID: 10448417.
    View in: PubMed
  62. Wang Y, Sheen VL, Macklis JD. Cortical interneurons upregulate neurotrophins in vivo in response to targeted apoptotic degeneration of neighboring pyramidal neurons. Exp Neurol. 1998 Dec; 154(2):389-402. PMID: 9878177.
    View in: PubMed
  63. Sheen VL, Macklis JD. Targeted neocortical cell death in adult mice guides migration and differentiation of transplanted embryonic neurons. J Neurosci. 1995 Dec; 15(12):8378-92. PMID: 8613770.
    View in: PubMed
  64. Sheen VL, Macklis JD. Apoptotic mechanisms in targeted neuronal cell death by chromophore-activated photolysis. Exp Neurol. 1994 Nov; 130(1):67-81. PMID: 7821398.
    View in: PubMed
  65. Sheen VL, Dreyer EB, Macklis JD. Calcium-mediated neuronal degeneration following singlet oxygen production. Neuroreport. 1992 Aug; 3(8):705-8. PMID: 1520860.
    View in: PubMed
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