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374 related items for PubMed ID: 29116375

  • 1. Uncoupling N-acetylaspartate from brain pathology: implications for Canavan disease gene therapy.
    von Jonquieres G, Spencer ZHT, Rowlands BD, Klugmann CB, Bongers A, Harasta AE, Parley KE, Cederholm J, Teahan O, Pickford R, Delerue F, Ittner LM, Fröhlich D, McLean CA, Don AS, Schneider M, Housley GD, Rae CD, Klugmann M.
    Acta Neuropathol; 2018 Jan; 135(1):95-113. PubMed ID: 29116375
    [Abstract] [Full Text] [Related]

  • 2. N-acetylaspartate supports the energetic demands of developmental myelination via oligodendroglial aspartoacylase.
    Francis JS, Wojtas I, Markov V, Gray SJ, McCown TJ, Samulski RJ, Bilaniuk LT, Wang DJ, De Vivo DC, Janson CG, Leone P.
    Neurobiol Dis; 2016 Dec; 96():323-334. PubMed ID: 27717881
    [Abstract] [Full Text] [Related]

  • 3. N-Acetylaspartate Synthase Deficiency Corrects the Myelin Phenotype in a Canavan Disease Mouse Model But Does Not Affect Survival Time.
    Maier H, Wang-Eckhardt L, Hartmann D, Gieselmann V, Eckhardt M.
    J Neurosci; 2015 Oct 28; 35(43):14501-16. PubMed ID: 26511242
    [Abstract] [Full Text] [Related]

  • 4. Brain Nat8l Knockdown Suppresses Spongiform Leukodystrophy in an Aspartoacylase-Deficient Canavan Disease Mouse Model.
    Bannerman P, Guo F, Chechneva O, Burns T, Zhu X, Wang Y, Kim B, Singhal NK, McDonough JA, Pleasure D.
    Mol Ther; 2018 Mar 07; 26(3):793-800. PubMed ID: 29456021
    [Abstract] [Full Text] [Related]

  • 5. Suppressing N-Acetyl-l-Aspartate Synthesis Prevents Loss of Neurons in a Murine Model of Canavan Leukodystrophy.
    Sohn J, Bannerman P, Guo F, Burns T, Miers L, Croteau C, Singhal NK, McDonough JA, Pleasure D.
    J Neurosci; 2017 Jan 11; 37(2):413-421. PubMed ID: 28077719
    [Abstract] [Full Text] [Related]

  • 6. Restoration of aspartoacylase activity in CNS neurons does not ameliorate motor deficits and demyelination in a model of Canavan disease.
    Klugmann M, Leichtlein CB, Symes CW, Serikawa T, Young D, During MJ.
    Mol Ther; 2005 May 11; 11(5):745-53. PubMed ID: 15851013
    [Abstract] [Full Text] [Related]

  • 7. Redirecting N-acetylaspartate metabolism in the central nervous system normalizes myelination and rescues Canavan disease.
    Gessler DJ, Li D, Xu H, Su Q, Sanmiguel J, Tuncer S, Moore C, King J, Matalon R, Gao G.
    JCI Insight; 2017 Feb 09; 2(3):e90807. PubMed ID: 28194442
    [Abstract] [Full Text] [Related]

  • 8. NAAG synthetase deficiency has only low influence on pathogenesis in a Canavan disease mouse model.
    Becker I, Wang-Eckhardt L, Eckhardt M.
    J Inherit Metab Dis; 2024 Mar 09; 47(2):230-243. PubMed ID: 38011891
    [Abstract] [Full Text] [Related]

  • 9. Adeno-associated virus-mediated aspartoacylase gene transfer to the brain of knockout mouse for canavan disease.
    Matalon R, Surendran S, Rady PL, Quast MJ, Campbell GA, Matalon KM, Tyring SK, Wei J, Peden CS, Ezell EL, Muzyczka N, Mandel RJ.
    Mol Ther; 2003 May 09; 7(5 Pt 1):580-7. PubMed ID: 12718900
    [Abstract] [Full Text] [Related]

  • 10. Nur7 is a nonsense mutation in the mouse aspartoacylase gene that causes spongy degeneration of the CNS.
    Traka M, Wollmann RL, Cerda SR, Dugas J, Barres BA, Popko B.
    J Neurosci; 2008 Nov 05; 28(45):11537-49. PubMed ID: 18987190
    [Abstract] [Full Text] [Related]

  • 11. Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model.
    Guo F, Bannerman P, Mills Ko E, Miers L, Xu J, Burns T, Li S, Freeman E, McDonough JA, Pleasure D.
    Ann Neurol; 2015 May 05; 77(5):884-8. PubMed ID: 25712859
    [Abstract] [Full Text] [Related]

  • 12. Are astrocytes the missing link between lack of brain aspartoacylase activity and the spongiform leukodystrophy in Canavan disease?
    Baslow MH, Guilfoyle DN.
    Neurochem Res; 2009 Sep 05; 34(9):1523-34. PubMed ID: 19319678
    [Abstract] [Full Text] [Related]

  • 13. Clinical protocol. Gene therapy of Canavan disease: AAV-2 vector for neurosurgical delivery of aspartoacylase gene (ASPA) to the human brain.
    Janson C, McPhee S, Bilaniuk L, Haselgrove J, Testaiuti M, Freese A, Wang DJ, Shera D, Hurh P, Rupin J, Saslow E, Goldfarb O, Goldberg M, Larijani G, Sharrar W, Liouterman L, Camp A, Kolodny E, Samulski J, Leone P.
    Hum Gene Ther; 2002 Jul 20; 13(11):1391-412. PubMed ID: 12162821
    [Abstract] [Full Text] [Related]

  • 14. Canavan disease: studies on the knockout mouse.
    Matalon R, Michals-Matalon K, Surendran S, Tyring SK.
    Adv Exp Med Biol; 2006 Jul 20; 576():77-93; discussion 361-3. PubMed ID: 16802706
    [Abstract] [Full Text] [Related]

  • 15. Canavan disease: a monogenic trait with complex genomic interaction.
    Surendran S, Michals-Matalon K, Quast MJ, Tyring SK, Wei J, Ezell EL, Matalon R.
    Mol Genet Metab; 2003 Jul 20; 80(1-2):74-80. PubMed ID: 14567959
    [Abstract] [Full Text] [Related]

  • 16. Knock-out mouse for Canavan disease: a model for gene transfer to the central nervous system.
    Matalon R, Rady PL, Platt KA, Skinner HB, Quast MJ, Campbell GA, Matalon K, Ceci JD, Tyring SK, Nehls M, Surendran S, Wei J, Ezell EL, Szucs S.
    J Gene Med; 2000 Jul 20; 2(3):165-75. PubMed ID: 10894262
    [Abstract] [Full Text] [Related]

  • 17. Canavan disease: a white matter disorder.
    Kumar S, Mattan NS, de Vellis J.
    Ment Retard Dev Disabil Res Rev; 2006 Jul 20; 12(2):157-65. PubMed ID: 16807907
    [Abstract] [Full Text] [Related]

  • 18. Astroglial conditional Slc13a3 knockout is therapeutic in murine Canavan leukodystrophy.
    Hull VL, Wang Y, McDonough J, Zhu M, Burns T, Al Ramel N, Dehghani A, Guo F, Pleasure D.
    Ann Clin Transl Neurol; 2024 Apr 20; 11(4):1059-1062. PubMed ID: 38282243
    [Abstract] [Full Text] [Related]

  • 19. Pathophysiology and Treatment of Canavan Disease.
    Pleasure D, Guo F, Chechneva O, Bannerman P, McDonough J, Burns T, Wang Y, Hull V.
    Neurochem Res; 2020 Mar 20; 45(3):561-565. PubMed ID: 30535831
    [No Abstract] [Full Text] [Related]

  • 20. Atypical clinical and radiological course of a patient with Canavan disease.
    Sarret C, Boespflug-Tanguy O, Rodriguez D.
    Metab Brain Dis; 2016 Apr 20; 31(2):475-9. PubMed ID: 26586007
    [Abstract] [Full Text] [Related]

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