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Busciglio, Jorge A

Title(s)Professor, Neurobiology & Behavior
Phone(949) 824-9075
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    The Role of Inflammation and NGF Dysfunction in the Evolution of AlzheimerDisease Pathology in Down syndrome
    NIH/NIA R01AG056850Apr 1, 2018 - Dec 31, 2022
    Role: Principal Investigator
    2nd International Conference of the Trisomy 21 Research Society
    NIH/NINDS R13NS100380Sep 1, 2016 - Aug 31, 2017
    Role: Principal Investigator
    Combinational pharmacotherapies for neuronal abnormalities in Down syndrome
    NIH/NICHD R21HD076456Jan 1, 2015 - Dec 31, 2016
    Role: Co-Principal Investigator
    iPSC from British and Danish dementias: new discovery tools for brain amyloidoses
    NIH/NIA R21AG044817Sep 30, 2013 - Jul 31, 2015
    Role: Co-Principal Investigator
    Mitochondrial Dysfunction in Down's Syndrome
    NIH/NICHD R01HD038466Mar 1, 2000 - Jun 30, 2011
    Role: Principal Investigator

    Collapse Bibliographic 
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    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Researchers can login to make corrections and additions, or contact us for help.
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    1. Reeves RH, Delabar J, Potier MC, Bhattacharyya A, Head E, Lemere C, Dekker AD, De Deyn P, Caviedes P, Dierssen M, Busciglio J. Paving the Way for Therapy: The Second International Conference of the Trisomy 21 Research Society. Mol Syndromol. 2019 Jan; 9(6):279-286. PMID: 30800043.
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    2. Zamponi E, Zamponi N, Coskun P, Quassollo G, Lorenzo A, Cannas SA, Pigino G, Chialvo DR, Gardiner K, Busciglio J, Helguera P. Nrf2 stabilization prevents critical oxidative damage in Down syndrome cells. Aging Cell. 2018 Oct; 17(5):e12812. PMID: 30028071.
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    3. Vogler EC, Flynn DT, Busciglio F, Bohannan RC, Tran A, Mahavongtrakul M, Busciglio JA. Low Cost Electrode Assembly for EEG Recordings in Mice. Front Neurosci. 2017; 11:629. PMID: 29184480.
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    4. Torres MD, Garcia O, Tang C, Busciglio J. Dendritic spine pathology and thrombospondin-1 deficits in Down syndrome. Free Radic Biol Med. 2018 01; 114:10-14. PMID: 28965914.
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    5. Coskun P, Helguera P, Nemati Z, Bohannan RC, Thomas J, Samuel SE, Argueta J, Doran E, Wallace DC, Lott IT, Busciglio J. Metabolic and Growth Rate Alterations in Lymphoblastic Cell Lines Discriminate Between Down Syndrome and Alzheimer's Disease. J Alzheimers Dis. 2017; 55(2):737-748. PMID: 27802222.
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    6. Delabar JM, Allinquant B, Bianchi D, Blumenthal T, Dekker A, Edgin J, O'Bryan J, Dierssen M, Potier MC, Wiseman F, Guedj F, Créau N, Reeves R, Gardiner K, Busciglio J. Changing Paradigms in Down Syndrome: The First International Conference of the Trisomy 21 Research Society. Mol Syndromol. 2016 Oct; 7(5):251-261. PMID: 27867340.
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    7. Peiris H, Duffield MD, Fadista J, Jessup CF, Kashmir V, Genders AJ, McGee SL, Martin AM, Saiedi M, Morton N, Carter R, Cousin MA, Kokotos AC, Oskolkov N, Volkov P, Hough TA, Fisher EM, Tybulewicz VL, Busciglio J, Coskun PE, Becker A, Belichenko PV, Mobley WC, Ryan MT, Chan JY, Laybutt DR, Coates PT, Yang S, Ling C, Groop L, Pritchard MA, Keating DJ. A Syntenic Cross Species Aneuploidy Genetic Screen Links RCAN1 Expression to ß-Cell Mitochondrial Dysfunction in Type 2 Diabetes. PLoS Genet. 2016 05; 12(5):e1006033. PMID: 27195491.
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    8. Iulita MF, Do Carmo S, Ower AK, Fortress AM, Flores Aguilar L, Hanna M, Wisniewski T, Granholm AC, Buhusi M, Busciglio J, Cuello AC. Nerve growth factor metabolic dysfunction in Down's syndrome brains. Brain. 2014 Mar; 137(Pt 3):860-72. PMID: 24519975.
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    9. Vogler EC, Busciglio J. Disruption of zinc neuromodulation by Aß oligomers: therapeutic implications. Curr Pharm Des. 2014; 20(15):2520-4. PMID: 23859544.
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    10. Sosa LJ, Postma NL, Estrada-Bernal A, Hanna M, Guo R, Busciglio J, Pfenninger KH. Dosage of amyloid precursor protein affects axonal contact guidance in Down syndrome. FASEB J. 2014 Jan; 28(1):195-205. PMID: 24036883.
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    11. Busciglio J, Capone G, O'Bryan J, O'Byran JP, Gardiner KJ. Down syndrome: genes, model systems, and progress towards pharmacotherapies and clinical trials for cognitive deficits. Cytogenet Genome Res. 2013; 141(4):260-71. PMID: 24008277.
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    12. Yeo M, Berglund K, Hanna M, Guo JU, Kittur J, Torres MD, Abramowitz J, Busciglio J, Gao Y, Birnbaumer L, Liedtke WB. Bisphenol A delays the perinatal chloride shift in cortical neurons by epigenetic effects on the Kcc2 promoter. Proc Natl Acad Sci U S A. 2013 Mar 12; 110(11):4315-20. PMID: 23440186.
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    13. Helguera P, Seiglie J, Rodriguez J, Hanna M, Helguera G, Busciglio J. Adaptive downregulation of mitochondrial function in down syndrome. Cell Metab. 2013 Jan 08; 17(1):132-40. PMID: 23312288.
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    14. Coskun PE, Busciglio J. Oxidative Stress and Mitochondrial Dysfunction in Down's Syndrome: Relevance to Aging and Dementia. Curr Gerontol Geriatr Res. 2012; 2012:383170. PMID: 22611387.
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    15. Tiano L, Busciglio J. Mitochondrial dysfunction and Down's syndrome: is there a role for coenzyme Q(10) ? Biofactors. 2011 Sep-Oct; 37(5):386-92. PMID: 22009852.
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    16. Garcia O, Torres M, Helguera P, Coskun P, Busciglio J. A role for thrombospondin-1 deficits in astrocyte-mediated spine and synaptic pathology in Down's syndrome. PLoS One. 2010 Dec 02; 5(12):e14200. PMID: 21152035; PMCID: PMC2996288.
    17. Rahman AS, Parvinjah S, Hanna MA, Helguera PR, Busciglio J. Cryopreservation of cortical tissue blocks for the generation of highly enriched neuronal cultures. J Vis Exp. 2010 Nov 11; (45). PMID: 21113113.
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    18. Deshpande A, Kawai H, Metherate R, Glabe CG, Busciglio J. A role for synaptic zinc in activity-dependent Abeta oligomer formation and accumulation at excitatory synapses. J Neurosci. 2009 Apr 01; 29(13):4004-15. PMID: 19339596.
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    19. Pigino G, Morfini G, Atagi Y, Deshpande A, Yu C, Jungbauer L, LaDu M, Busciglio J, Brady S. Disruption of fast axonal transport is a pathogenic mechanism for intraneuronal amyloid beta. Proc Natl Acad Sci U S A. 2009 Apr 07; 106(14):5907-12. PMID: 19321417.
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    20. Resende R, Moreira PI, Proença T, Deshpande A, Busciglio J, Pereira C, Oliveira CR. Brain oxidative stress in a triple-transgenic mouse model of Alzheimer disease. Free Radic Biol Med. 2008 Jun 15; 44(12):2051-7. PMID: 18423383.
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    21. Deshpande A, Win KM, Busciglio J. Tau isoform expression and regulation in human cortical neurons. FASEB J. 2008 Jul; 22(7):2357-67. PMID: 18263702.
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    22. Busciglio J, Pelsman A, Helguera P, Ashur-Fabian O, Pinhasov A, Brenneman DE, Gozes I. NAP and ADNF-9 protect normal and Down's syndrome cortical neurons from oxidative damage and apoptosis. Curr Pharm Des. 2007; 13(11):1091-8. PMID: 17430172.
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    23. Lott IT, Head E, Doran E, Busciglio J. Beta-amyloid, oxidative stress and down syndrome. Curr Alzheimer Res. 2006 Dec; 3(5):521-8. PMID: 17168651.
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    24. Heredia L, Helguera P, de Olmos S, Kedikian G, Solá Vigo F, LaFerla F, Staufenbiel M, de Olmos J, Busciglio J, Cáceres A, Lorenzo A. Phosphorylation of actin-depolymerizing factor/cofilin by LIM-kinase mediates amyloid beta-induced degeneration: a potential mechanism of neuronal dystrophy in Alzheimer's disease. J Neurosci. 2006 Jun 14; 26(24):6533-42. PMID: 16775141.
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    25. Deshpande A, Mina E, Glabe C, Busciglio J. Different conformations of amyloid beta induce neurotoxicity by distinct mechanisms in human cortical neurons. J Neurosci. 2006 May 31; 26(22):6011-8. PMID: 16738244.
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    26. Helguera P, Pelsman A, Pigino G, Wolvetang E, Head E, Busciglio J. ets-2 promotes the activation of a mitochondrial death pathway in Down's syndrome neurons. J Neurosci. 2005 Mar 02; 25(9):2295-303. PMID: 15745955.
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    27. Heredia L, Lin R, Vigo FS, Kedikian G, Busciglio J, Lorenzo A. Deposition of amyloid fibrils promotes cell-surface accumulation of amyloid beta precursor protein. Neurobiol Dis. 2004 Aug; 16(3):617-29. PMID: 15262274.
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    28. Goldstein L, Ashford JW, Busciglio J, Crutcher K, Gouras G, Kinoshita J, Mandelkow E, Sudol M. Live discussion. From here to there: AbetaPP as an axonal transport receptor--how could this explain neurodegeneration in AD. 15 July 2002. J Alzheimers Dis. 2003 Dec; 5(6):483-9. PMID: 14765517.
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    29. Wolvetang EJ, Bradfield OM, Hatzistavrou T, Crack PJ, Busciglio J, Kola I, Hertzog PJ. Overexpression of the chromosome 21 transcription factor Ets2 induces neuronal apoptosis. Neurobiol Dis. 2003 Dec; 14(3):349-56. PMID: 14678752.
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    30. Pigino G, Morfini G, Pelsman A, Mattson MP, Brady ST, Busciglio J. Alzheimer's presenilin 1 mutations impair kinesin-based axonal transport. J Neurosci. 2003 Jun 01; 23(11):4499-508. PMID: 12805290.
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    31. Pelsman A, Hoyo-Vadillo C, Gudasheva TA, Seredenin SB, Ostrovskaya RU, Busciglio J. GVS-111 prevents oxidative damage and apoptosis in normal and Down's syndrome human cortical neurons. Int J Dev Neurosci. 2003 May; 21(3):117-24. PMID: 12711349.
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    32. Wolvetang EJ, Wilson TJ, Sanij E, Busciglio J, Hatzistavrou T, Seth A, Hertzog PJ, Kola I. ETS2 overexpression in transgenic models and in Down syndrome predisposes to apoptosis via the p53 pathway. Hum Mol Genet. 2003 Feb 01; 12(3):247-55. PMID: 12554679.
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    33. Grace EA, Busciglio J. Aberrant activation of focal adhesion proteins mediates fibrillar amyloid beta-induced neuronal dystrophy. J Neurosci. 2003 Jan 15; 23(2):493-502. PMID: 12533609.
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    34. Busciglio J, Pelsman A, Wong C, Pigino G, Yuan M, Mori H, Yankner BA. Altered metabolism of the amyloid beta precursor protein is associated with mitochondrial dysfunction in Down's syndrome. Neuron. 2002 Feb 28; 33(5):677-88. PMID: 11879646.
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    35. Morfini G, Pigino G, Beffert U, Busciglio J, Brady ST. Fast axonal transport misregulation and Alzheimer's disease. Neuromolecular Med. 2002; 2(2):89-99. PMID: 12428805.
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    36. Grace EA, Rabiner CA, Busciglio J. Characterization of neuronal dystrophy induced by fibrillar amyloid beta: implications for Alzheimer's disease. Neuroscience. 2002; 114(1):265-73. PMID: 12207971.
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    37. Pigino G, Pelsman A, Mori H, Busciglio J. Presenilin-1 mutations reduce cytoskeletal association, deregulate neurite growth, and potentiate neuronal dystrophy and tau phosphorylation. J Neurosci. 2001 Feb 01; 21(3):834-42. PMID: 11157069.
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    38. Kerkovich DM, Sapp D, Weidenheim K, Brosnan CF, Pfeiffer SE, Yeh HH, Busciglio J. Fetal human cortical neurons grown in culture: morphological differentiation, biochemical correlates and development of electrical activity. Int J Dev Neurosci. 1999 Jul; 17(4):347-56. PMID: 10479069.
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    39. Busciglio J, Andersen JK, Schipper HM, Gilad GM, McCarty R, Marzatico F, Toussaint O. Stress, aging, and neurodegenerative disorders. Molecular mechanisms. Ann N Y Acad Sci. 1998 Jun 30; 851:429-43. PMID: 9668637.
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    40. Shi B, Raina J, Lorenzo A, Busciglio J, Gabuzda D. Neuronal apoptosis induced by HIV-1 Tat protein and TNF-alpha: potentiation of neurotoxicity mediated by oxidative stress and implications for HIV-1 dementia. J Neurovirol. 1998 Jun; 4(3):281-90. PMID: 9639071.
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    41. Busciglio J, Hartmann H, Lorenzo A, Wong C, Baumann K, Sommer B, Staufenbiel M, Yankner BA. Neuronal localization of presenilin-1 and association with amyloid plaques and neurofibrillary tangles in Alzheimer's disease. J Neurosci. 1997 Jul 01; 17(13):5101-7. PMID: 9185547.
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    42. Hartmann H, Busciglio J, Baumann KH, Staufenbiel M, Yankner BA. Developmental regulation of presenilin-1 processing in the brain suggests a role in neuronal differentiation. J Biol Chem. 1997 Jun 06; 272(23):14505-8. PMID: 9169406.
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    43. He J, deCastro CM, Vandenbark GR, Busciglio J, Gabuzda D. Astrocyte apoptosis induced by HIV-1 transactivation of the c-kit protooncogene. Proc Natl Acad Sci U S A. 1997 Apr 15; 94(8):3954-9. PMID: 9108086.
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    44. He J, Chen Y, Farzan M, Choe H, Ohagen A, Gartner S, Busciglio J, Yang X, Hofmann W, Newman W, Mackay CR, Sodroski J, Gabuzda D. CCR3 and CCR5 are co-receptors for HIV-1 infection of microglia. Nature. 1997 Feb 13; 385(6617):645-9. PMID: 9024664.
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    45. Shi B, De Girolami U, He J, Wang S, Lorenzo A, Busciglio J, Gabuzda D. Apoptosis induced by HIV-1 infection of the central nervous system. J Clin Invest. 1996 Nov 01; 98(9):1979-90. PMID: 8903316.
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    46. Busciglio J, Yankner BA. Apoptosis and increased generation of reactive oxygen species in Down's syndrome neurons in vitro. Nature. 1995 Dec 21-28; 378(6559):776-9. PMID: 8524410.
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    47. Martin BL, Schrader-Fischer G, Busciglio J, Duke M, Paganetti P, Yankner BA. Intracellular accumulation of beta-amyloid in cells expressing the Swedish mutant amyloid precursor protein. J Biol Chem. 1995 Nov 10; 270(45):26727-30. PMID: 7592902.
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    48. Busciglio J, Lorenzo A, Yeh J, Yankner BA. beta-amyloid fibrils induce tau phosphorylation and loss of microtubule binding. Neuron. 1995 Apr; 14(4):879-88. PMID: 7718249.
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    49. Gabuzda D, Busciglio J, Chen LB, Matsudaira P, Yankner BA. Inhibition of energy metabolism alters the processing of amyloid precursor protein and induces a potentially amyloidogenic derivative. J Biol Chem. 1994 May 06; 269(18):13623-8. PMID: 8175797.
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    50. Gabuzda D, Busciglio J, Yankner BA. Inhibition of beta-amyloid production by activation of protein kinase C. J Neurochem. 1993 Dec; 61(6):2326-9. PMID: 8245986.
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    51. Busciglio J, Yeh J, Yankner BA. beta-Amyloid neurotoxicity in human cortical culture is not mediated by excitotoxins. J Neurochem. 1993 Oct; 61(4):1565-8. PMID: 8377009.
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    52. Busciglio J, Gabuzda DH, Matsudaira P, Yankner BA. Generation of beta-amyloid in the secretory pathway in neuronal and nonneuronal cells. Proc Natl Acad Sci U S A. 1993 Mar 01; 90(5):2092-6. PMID: 8446635.
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    53. Busciglio J, Lorenzo A, Yankner BA. Methodological variables in the assessment of beta amyloid neurotoxicity. Neurobiol Aging. 1992 Sep-Oct; 13(5):609-12. PMID: 1461350.
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    54. Kowall NW, Beal MF, Busciglio J, Duffy LK, Yankner BA. An in vivo model for the neurodegenerative effects of beta amyloid and protection by substance P. Proc Natl Acad Sci U S A. 1991 Aug 15; 88(16):7247-51. PMID: 1714596.
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    55. Arregui C, Busciglio J, Caceres A, Barra HS. Tyrosinated and detyrosinated microtubules in axonal processes of cerebellar macroneurons grown in culture. J Neurosci Res. 1991 Feb; 28(2):171-81. PMID: 1674546.
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    56. Gravotta D, Ferreira A, Busciglio J, Caceres A, Landa CA, Maccioni HJ. Coexpression of lactosyl and gangliotetraosyl gangliosides in rat cerebellar radial glial cells in culture. J Neurosci Res. 1990 Feb; 25(2):214-22. PMID: 2319630.
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    57. Ferreira A, Busciglio J, Landa C, Caceres A. Ganglioside-enhanced neurite growth: evidence for a selective induction of high-molecular-weight MAP-2. J Neurosci. 1990 Jan; 10(1):293-302. PMID: 2153774.
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    58. Ferreira A, Busciglio J, Cáceres A. Microtubule formation and neurite growth in cerebellar macroneurons which develop in vitro: evidence for the involvement of the microtubule-associated proteins, MAP-1a, HMW-MAP2 and Tau. Brain Res Dev Brain Res. 1989 Oct 01; 49(2):215-28. PMID: 2509111.
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    59. Cáceres A, Busciglio J, Ferreira A, Steward O. An immunocytochemical and biochemical study of the microtubule-associated protein MAP-2 during post-lesion dendritic remodeling in the central nervous system of adult rats. Brain Res. 1988 Jun; 427(3):233-46. PMID: 3042088.
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    60. Busciglio J, Ferreira A, Steward O, Cáceres A. An immunocytochemical and biochemical study of the microtubule-associated protein Tau during post-lesion afferent reorganization in the hippocampus of adult rats. Brain Res. 1987 Sep 01; 419(1-2):244-52. PMID: 3119145.
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    61. Ferreira A, Busciglio J, Cáceres A. An immunocytochemical analysis of the ontogeny of the microtubule-associated proteins MAP-2 and Tau in the nervous system of the rat. Brain Res. 1987 Jul; 431(1):9-31. PMID: 3113673.
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