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JAMES A. MASTRIANNI

TitleProfessor
InstitutionUniversity of Chicago
DepartmentNeurology
AddressChicago IL 60637
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    Collapse Overview 

    Collapse Biography 
    Collapse education and training
    Albany College of Pharmacy, Albany N.Y.B.S. Pharmacy06/1980Chemistry/Pharmacy
    University of North Carolina, Greenville, N.C.Ph.D.06/1985Pharmacology
    McGill University School of Medicine, Montreal, Quebec, CanadaM.D.C.M.06/1989Medicine
    University of Pennsylvania, Philadelphia, PA06/1993Residency in Neurology
    UCSF, San Francisco11/1998postdoc - molecular genetics prion diseases

    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. Cali I, Mikhail F, Qin K, Gregory C, Solanki A, Martinez MC, Zhao L, Appleby B, Gambetti P, Norstrom E, Mastrianni JA. Impaired transmissibility of atypical prions from genetic CJDG114V. Neurol Genet. 2018 Aug; 4(4):e253. PMID: 30109268.
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    2. Gu H, Kirchhein Y, Zhu T, Zhao G, Peng H, Du E, Liu J, Mastrianni JA, Farlow MR, Dodel R, Du Y. IVIG Delays Onset in a Mouse Model of Gerstmann-Sträussler-Scheinker Disease. Mol Neurobiol. 2018 Jul 19. PMID: 30027340.
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    3. Klionsky DJ, Abdelmohsen K, Abe A, Abedin MJ, Abeliovich H, Acevedo Arozena A, Adachi H, Adams CM, Adams PD, Adeli K, Adhihetty PJ, Adler SG, Agam G, Agarwal R, Aghi MK, Agnello M, Agostinis P, Aguilar PV, Aguirre-Ghiso J, Airoldi EM, Ait-Si-Ali S, Akematsu T, Akporiaye ET, Al-Rubeai M, Albaiceta GM, Albanese C, Albani D, Albert ML, Aldudo J, Algül H, Alirezaei M, Alloza I, Almasan A, Almonte-Beceril M, Alnemri ES, Alonso C, Altan-Bonnet N, Altieri DC, Alvarez S, Alvarez-Erviti L, Alves S, Amadoro G, Amano A, Amantini C, Ambrosio S, Amelio I, Amer AO, Amessou M, Amon A, An Z, Anania FA, Andersen SU, Andley UP, Andreadi CK, Andrieu-Abadie N, Anel A, Ann DK, Anoopkumar-Dukie S, Antonioli M, Aoki H, Apostolova N, Aquila S, Aquilano K, Araki K, Arama E, Aranda A, Araya J, Arcaro A, Arias E, Arimoto H, Ariosa AR, Armstrong JL, Arnould T, Arsov I, Asanuma K, Askanas V, Asselin E, Atarashi R, Atherton SS, Atkin JD, Attardi LD, Auberger P, Auburger G, Aurelian L, Autelli R, Avagliano L, Avantaggiati ML, Avrahami L, Awale S, Azad N, Bachetti T, Backer JM, Bae DH, Bae JS, Bae ON, Bae SH, Baehrecke EH, Baek SH, Baghdiguian S, Bagniewska-Zadworna A, Bai H, Bai J, Bai XY, Bailly Y, Balaji KN, Balduini W, Ballabio A, Balzan R, Banerjee R, Bánhegyi G, Bao H, Barbeau B, Barrachina MD, Barreiro E, Bartel B, Bartolomé A, Bassham DC, Bassi MT, Bast RC, Basu A, Batista MT, Batoko H, Battino M, Bauckman K, Baumgarner BL, Bayer KU, Beale R, Beaulieu JF, Beck GR, Becker C, Beckham JD, Bédard PA, Bednarski PJ, Begley TJ, Behl C, Behrends C, Behrens GM, Behrns KE, Bejarano E, Belaid A, Belleudi F, Bénard G, Berchem G, Bergamaschi D, Bergami M, Berkhout B, Berliocchi L, Bernard A, Bernard M, Bernassola F, Bertolotti A, Bess AS, Besteiro S, Bettuzzi S, Bhalla S, Bhattacharyya S, Bhutia SK, Biagosch C, Bianchi MW, Biard-Piechaczyk M, Billes V, Bincoletto C, Bingol B, Bird SW, Bitoun M, Bjedov I, Blackstone C, Blanc L, Blanco GA, Blomhoff HK, Boada-Romero E, Böckler S, Boes M, Boesze-Battaglia K, Boise LH, Bolino A, Boman A, Bonaldo P, Bordi M, Bosch J, Botana LM, Botti J, Bou G, Bouché M, Bouchecareilh M, Boucher MJ, Boulton ME, Bouret SG, Boya P, Boyer-Guittaut M, Bozhkov PV, Brady N, Braga VM, Brancolini C, Braus GH, Bravo-San Pedro JM, Brennan LA, Bresnick EH, Brest P, Bridges D, Bringer MA, Brini M, Brito GC, Brodin B, Brookes PS, Brown EJ, Brown K, Broxmeyer HE, Bruhat A, Brum PC, Brumell JH, Brunetti-Pierri N, Bryson-Richardson RJ, Buch S, Buchan AM, Budak H, Bulavin DV, Bultman SJ, Bultynck G, Bumbasirevic V, Burelle Y, Burke RE, Burmeister M, Bütikofer P, Caberlotto L, Cadwell K, Cahova M, Cai D, Cai J, Cai Q, Calatayud S, Camougrand N, Campanella M, Campbell GR, Campbell M, Campello S, Candau R, Caniggia I, Cantoni L, Cao L, Caplan AB, Caraglia M, Cardinali C, Cardoso SM, Carew JS, Carleton LA, Carlin CR, Carloni S, Carlsson SR, Carmona-Gutierrez D, Carneiro LA, Carnevali O, Carra S, Carrier A, Carroll B, Casas C, Casas J, Cassinelli G, Castets P, Castro-Obregon S, Cavallini G, Ceccherini I, Cecconi F, Cederbaum AI, Ceña V, Cenci S, Cerella C, Cervia D, Cetrullo S, Chaachouay H, Chae HJ, Chagin AS, Chai CY, Chakrabarti G, Chamilos G, Chan EY, Chan MT, Chandra D, Chandra P, Chang CP, Chang RC, Chang TY, Chatham JC, Chatterjee S, Chauhan S, Che Y, Cheetham ME, Cheluvappa R, Chen CJ, Chen G, Chen GC, Chen G, Chen H, Chen JW, Chen JK, Chen M, Chen M, Chen P, Chen Q, Chen Q, Chen SD, Chen S, Chen SS, Chen W, Chen WJ, Chen WQ, Chen W, Chen X, Chen YH, Chen YG, Chen Y, Chen Y, Chen Y, Chen YJ, Chen YQ, Chen Y, Chen Z, Chen Z, Cheng A, Cheng CH, Cheng H, Cheong H, Cherry S, Chesney J, Cheung CH, Chevet E, Chi HC, Chi SG, Chiacchiera F, Chiang HL, Chiarelli R, Chiariello M, Chieppa M, Chin LS, Chiong M, Chiu GN, Cho DH, Cho SG, Cho WC, Cho YY, Cho YS, Choi AM, Choi EJ, Choi EK, Choi J, Choi ME, Choi SI, Chou TF, Chouaib S, Choubey D, Choubey V, Chow KC, Chowdhury K, Chu CT, Chuang TH, Chun T, Chung H, Chung T, Chung YL, Chwae YJ, Cianfanelli V, et al. Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition). Autophagy. 2016; 12(1):1-222. PMID: 26799652.
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    4. Cortes CJ, Qin K, Norstrom EM, Green WN, Bindokas VP, Mastrianni JA. Early Delivery of Misfolded PrP from ER to Lysosomes by Autophagy. Int J Cell Biol. 2013; 2013:560421. PMID: 24454378.
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    5. Solodkin A, Chen EE, Van Hoesen GW, Heimer L, Shereen A, Kruggel F, Mastrianni J. In vivo parahippocampal white matter pathology as a biomarker of disease progression to Alzheimer's disease. J Comp Neurol. 2013 Dec 15; 521(18):4300-17. PMID: 23839862.
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    6. Rezania K, Pytel P, Smit LJ, Mastrianni J, Dina MA, Highsmith WE, Dogan A. Systemic transthyretin amyloidosis in a patient with bent spine syndrome. Amyloid. 2013 Jun; 20(2):131-4. PMID: 23638719.
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    7. Fan Y, McGowan S, Rubeiz H, Wollmann R, Javed A, Mastrianni J. Acute encephalopathy as the initial manifestation of CADASIL. Neurol Clin Pract. 2012 Dec; 2(4):359-361. PMID: 23634379.
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    8. Cortes CJ, Qin K, Cook J, Solanki A, Mastrianni JA. Rapamycin delays disease onset and prevents PrP plaque deposition in a mouse model of Gerstmann-Sträussler-Scheinker disease. J Neurosci. 2012 Sep 05; 32(36):12396-405. PMID: 22956830.
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    9. Leggett C, McGehee DS, Mastrianni J, Yang W, Bai T, Brorson JR. Tunicamycin produces TDP-43 cytoplasmic inclusions in cultured brain organotypic slices. J Neurol Sci. 2012 Jun 15; 317(1-2):66-73. PMID: 22459357.
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    10. Jayadev S, Nochlin D, Poorkaj P, Steinbart EJ, Mastrianni JA, Montine TJ, Ghetti B, Schellenberg GD, Bird TD, Leverenz JB. Familial prion disease with Alzheimer disease-like tau pathology and clinical phenotype. Ann Neurol. 2011 Apr; 69(4):712-20. PMID: 21416485.
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    11. Barria MA, Telling GC, Gambetti P, Mastrianni JA, Soto C. Generation of a new form of human PrP(Sc) in vitro by interspecies transmission from cervid prions. J Biol Chem. 2011 Mar 04; 286(9):7490-5. PMID: 21209079.
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    12. Brown K, Mastrianni JA. The prion diseases. J Geriatr Psychiatry Neurol. 2010 Dec; 23(4):277-98. PMID: 20938044.
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    13. Zou WQ, Puoti G, Xiao X, Yuan J, Qing L, Cali I, Shimoji M, Langeveld JP, Castellani R, Notari S, Crain B, Schmidt RE, Geschwind M, Dearmond SJ, Cairns NJ, Dickson D, Honig L, Torres JM, Mastrianni J, Capellari S, Giaccone G, Belay ED, Schonberger LB, Cohen M, Perry G, Kong Q, Parchi P, Tagliavini F, Gambetti P. Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein. Ann Neurol. 2010 Aug; 68(2):162-72. PMID: 20695009.
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    14. Mastrianni JA. The genetics of prion diseases. Genet Med. 2010 Apr; 12(4):187-95. PMID: 20216075.
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    15. Mallik S, Yang W, Norstrom EM, Mastrianni JA. Live cell fluorescence resonance energy transfer predicts an altered molecular association of heterologous PrPSc with PrPC. J Biol Chem. 2010 Mar 19; 285(12):8967-75. PMID: 20086009.
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    16. Yang W, Cook J, Rassbach B, Lemus A, DeArmond SJ, Mastrianni JA. A New Transgenic Mouse Model of Gerstmann-Straussler-Scheinker Syndrome Caused by the A117V Mutation of PRNP. J Neurosci. 2009 Aug 12; 29(32):10072-80. PMID: 19675240.
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    17. Tunnell E, Wollman R, Mallik S, Cortes CJ, Dearmond SJ, Mastrianni JA. A novel PRNP-P105S mutation associated with atypical prion disease and a rare PrPSc conformation. Neurology. 2008 Oct 28; 71(18):1431-8. PMID: 18955686.
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    18. Abalos GC, Cruite JT, Bellon A, Hemmers S, Akagi J, Mastrianni JA, Williamson RA, Solforosi L. Identifying key components of the PrPC-PrPSc replicative interface. J Biol Chem. 2008 Dec 05; 283(49):34021-8. PMID: 18826953.
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    19. Gambetti P, Dong Z, Yuan J, Xiao X, Zheng M, Alshekhlee A, Castellani R, Cohen M, Barria MA, Gonzalez-Romero D, Belay ED, Schonberger LB, Marder K, Harris C, Burke JR, Montine T, Wisniewski T, Dickson DW, Soto C, Hulette CM, Mastrianni JA, Kong Q, Zou WQ. A novel human disease with abnormal prion protein sensitive to protease. Ann Neurol. 2008 Jun; 63(6):697-708. PMID: 18571782.
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    20. Norstrom EM, Ciaccio MF, Rassbach B, Wollmann R, Mastrianni JA. Cytosolic prion protein toxicity is independent of cellular prion protein expression and prion propagation. J Virol. 2007 Mar; 81(6):2831-7. PMID: 17182694.
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    21. Norstrom EM, Mastrianni JA. The charge structure of helix 1 in the prion protein regulates conversion to pathogenic PrPSc. J Virol. 2006 Sep; 80(17):8521-9. PMID: 16912302.
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    22. Li X, Rowland LP, Mitsumoto H, Przedborski S, Bird TD, Schellenberg GD, Peskind E, Johnson N, Siddique T, Mesulam MM, Weintraub S, Mastrianni JA. Prion protein codon 129 genotype prevalence is altered in primary progressive aphasia. Ann Neurol. 2005 Dec; 58(6):858-64. PMID: 16315279.
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    23. Liebman SW, Mastrianni JA. Tracking the elusive prion. Trends Mol Med. 2005 Oct; 11(10):439-41. PMID: 16150640.
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    24. Norstrom EM, Mastrianni JA. The AGAAAAGA palindrome in PrP is required to generate a productive PrPSc-PrPC complex that leads to prion propagation. J Biol Chem. 2005 Jul 22; 280(29):27236-43. PMID: 15917252.
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    25. Mastrianni JA, Roos RP. Wrinkles and folds of the prion protein. Neurology. 2003 Nov 11; 61(9):1168-9. PMID: 14610113.
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    26. Korth C, Kaneko K, Groth D, Heye N, Telling G, Mastrianni J, Parchi P, Gambetti P, Will R, Ironside J, Heinrich C, Tremblay P, DeArmond SJ, Prusiner SB. Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene. Proc Natl Acad Sci U S A. 2003 Apr 15; 100(8):4784-9. PMID: 12684540.
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    27. Mastrianni J, Roos RP. "Out, damned spot! out, I say!...": issues related to prion decontamination. Neurology. 2002 Aug 27; 59(4):488-9. PMID: 12196639.
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    28. Mastrianni JA, Capellari S, Telling GC, Han D, Bosque P, Prusiner SB, DeArmond SJ. Inherited prion disease caused by the V210I mutation: transmission to transgenic mice. Neurology. 2001 Dec 26; 57(12):2198-205. PMID: 11756597.
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    29. Mastrianni JA, Roos RP. The prion diseases. Semin Neurol. 2000; 20(3):337-52. PMID: 11051298.
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    30. Worrall BB, Rowland LP, Chin SS, Mastrianni JA. Amyotrophy in prion diseases. Arch Neurol. 2000 Jan; 57(1):33-8. PMID: 10634430.
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    31. Poduslo SE, Yin X, Hargis J, Brumback RA, Mastrianni JA, Schwankhaus J. A familial case of Alzheimer's disease without tau pathology may be linked with chromosome 3 markers. Hum Genet. 1999 Jul-Aug; 105(1-2):32-7. PMID: 10480352.
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    32. Mastrianni JA, Nixon R, Layzer R, Telling GC, Han D, DeArmond SJ, Prusiner SB. Prion protein conformation in a patient with sporadic fatal insomnia. N Engl J Med. 1999 May 27; 340(21):1630-8. PMID: 10341275.
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    33. Patry D, Curry B, Easton D, Mastrianni JA, Hogan DB. Creutzfeldt-Jakob disease (CJD) after blood product transfusion from a donor with CJD. Neurology. 1998 Jun; 50(6):1872-3. PMID: 9633747.
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    34. Hegde RS, Mastrianni JA, Scott MR, DeFea KA, Tremblay P, Torchia M, DeArmond SJ, Prusiner SB, Lingappa VR. A transmembrane form of the prion protein in neurodegenerative disease. Science. 1998 Feb 06; 279(5352):827-34. PMID: 9452375.
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    35. Mastrianni JA. The prion diseases: Creutzfeldt-Jakob, Gerstmann-Sträussler-Scheinker, and related disorders. J Geriatr Psychiatry Neurol. 1998; 11(2):78-97. PMID: 9877529.
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    36. Schuff N, Amend D, Ezekiel F, Steinman SK, Tanabe J, Norman D, Jagust W, Kramer JH, Mastrianni JA, Fein G, Weiner MW. Changes of hippocampal N-acetyl aspartate and volume in Alzheimer's disease. A proton MR spectroscopic imaging and MRI study. Neurology. 1997 Dec; 49(6):1513-21. PMID: 9409338.
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    37. MacGowan DJ, Delanty N, Petito F, Edgar M, Mastrianni J, DeArmond SJ. Isolated myoclonic alien hand as the sole presentation of pathologically established Creutzfeldt-Jakob disease: a report of two patients. J Neurol Neurosurg Psychiatry. 1997 Sep; 63(3):404-7. PMID: 9328266.
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    38. Telling GC, Parchi P, DeArmond SJ, Cortelli P, Montagna P, Gabizon R, Mastrianni J, Lugaresi E, Gambetti P, Prusiner SB. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science. 1996 Dec 20; 274(5295):2079-82. PMID: 8953038.
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    39. Mastrianni JA, Iannicola C, Myers RM, DeArmond S, Prusiner SB. Mutation of the prion protein gene at codon 208 in familial Creutzfeldt-Jakob disease. Neurology. 1996 Nov; 47(5):1305-12. PMID: 8909447.
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    40. Mastrianni JA, Curtis MT, Oberholtzer JC, Da Costa MM, DeArmond S, Prusiner SB, Garbern JY. Prion disease (PrP-A117V) presenting with ataxia instead of dementia. Neurology. 1995 Nov; 45(11):2042-50. PMID: 7501157.
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    41. Telling GC, Scott M, Mastrianni J, Gabizon R, Torchia M, Cohen FE, DeArmond SJ, Prusiner SB. Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell. 1995 Oct 06; 83(1):79-90. PMID: 7553876.
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    42. Spudich S, Mastrianni JA, Wrensch M, Gabizon R, Meiner Z, Kahana I, Rosenmann H, Kahana E, Prusiner SB. Complete penetrance of Creutzfeldt-Jakob disease in Libyan Jews carrying the E200K mutation in the prion protein gene. Mol Med. 1995 Sep; 1(6):607-13. PMID: 8529127.
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    43. Mastrianni JA, Galetta SL, Raps EC, Liu GT, Volpe NJ. Isolated fascicular abducens nerve palsy and Lyme disease. J Neuroophthalmol. 1994 Mar; 14(1):2-5. PMID: 8032474.
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    44. Coloski D, Mastrianni J, Dube R, Brown LH. Continuous arteriovenous hemofiltration patient: nursing care plan. Dimens Crit Care Nurs. 1990 May-Jun; 9(3):130-42. PMID: 2340783.
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    45. Mastrianni JA, Abbott FV, Kunos G. Activation of central mu-opioid receptors is involved in clonidine analgesia in rats. Brain Res. 1989 Feb 13; 479(2):283-9. PMID: 2538210.
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    46. Mastrianni JA, Palkovits M, Kunos G. Activation of brainstem endorphinergic neurons causes cardiovascular depression and facilitates baroreflex bradycardia. Neuroscience. 1989; 33(3):559-66. PMID: 2636709.
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    47. Kunos G, Mosqueda-Garcia R, Mastrianni JA, Abbott FV. Endorphinergic mechanism in the central cardiovascular and analgesic effects of clonidine. Can J Physiol Pharmacol. 1987 Aug; 65(8):1624-32. PMID: 3690393.
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    48. Mastrianni JA, Ingenito AJ. On the relationship between clonidine hypotension and brain beta-endorphin in the spontaneously hypertensive rat: studies with alpha adrenergic and opiate blockers. J Pharmacol Exp Ther. 1987 Jul; 242(1):378-87. PMID: 3039113.
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    49. Mastrianni JA, Harris TM, Ingenito AJ. An intracerebroventricular perfusion system developed for the study of centrally acting antihypertensive drugs in the rat. J Pharmacol Methods. 1986 Aug; 16(1):63-72. PMID: 3747547.
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    50. Mastrianni JA, Ingenito AJ. Acute tolerance to clonidine hypotension and bradycardia in normotensive and hypertensive rats. Pharmacol Res Commun. 1985 Sep; 17(9):865-72. PMID: 4059320.
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    51. Garris DR, Smith-West C, Mastrianni JA. A cryostat-freeze drying technique for enhanced visualization of catecholamine/indoleamine-containing neurons. Neurosci Lett. 1983 Aug 29; 39(2):113-8. PMID: 6355908.
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