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255 related items for PubMed ID: 22031903
21. Synergistic effects of treating the spinal cord and brain in CLN1 disease. Shyng C, Nelvagal HR, Dearborn JT, Tyynelä J, Schmidt RE, Sands MS, Cooper JD. Proc Natl Acad Sci U S A; 2017 Jul 18; 114(29):E5920-E5929. PubMed ID: 28673981 [Abstract] [Full Text] [Related]
22. Cln1 gene disruption in mice reveals a common pathogenic link between two of the most lethal childhood neurodegenerative lysosomal storage disorders. Chandra G, Bagh MB, Peng S, Saha A, Sarkar C, Moralle M, Zhang Z, Mukherjee AB. Hum Mol Genet; 2015 Oct 01; 24(19):5416-32. PubMed ID: 26160911 [Abstract] [Full Text] [Related]
23. In a mouse model of INCL reduced S-palmitoylation of cytosolic thioesterase APT1 contributes to microglia proliferation and neuroinflammation. Sadhukhan T, Bagh MB, Appu AP, Mondal A, Zhang W, Liu A, Mukherjee AB. J Inherit Metab Dis; 2021 Jul 01; 44(4):1051-1069. PubMed ID: 33739454 [Abstract] [Full Text] [Related]
24. Stop codon read-through with PTC124 induces palmitoyl-protein thioesterase-1 activity, reduces thioester load and suppresses apoptosis in cultured cells from INCL patients. Sarkar C, Zhang Z, Mukherjee AB. Mol Genet Metab; 2011 Nov 01; 104(3):338-45. PubMed ID: 21704547 [Abstract] [Full Text] [Related]
25. Non-invasive assessment of retinal alterations in mouse models of infantile and juvenile neuronal ceroid lipofuscinosis by spectral domain optical coherence tomography. Groh J, Stadler D, Buttmann M, Martini R. Acta Neuropathol Commun; 2014 May 10; 2():54. PubMed ID: 24887158 [Abstract] [Full Text] [Related]
26. Neuroprotection of host cells by human central nervous system stem cells in a mouse model of infantile neuronal ceroid lipofuscinosis. Tamaki SJ, Jacobs Y, Dohse M, Capela A, Cooper JD, Reitsma M, He D, Tushinski R, Belichenko PV, Salehi A, Mobley W, Gage FH, Huhn S, Tsukamoto AS, Weissman IL, Uchida N. Cell Stem Cell; 2009 Sep 04; 5(3):310-9. PubMed ID: 19733542 [Abstract] [Full Text] [Related]
30. Pathogenesis and therapies for infantile neuronal ceroid lipofuscinosis (infantile CLN1 disease). Hawkins-Salsbury JA, Cooper JD, Sands MS. Biochim Biophys Acta; 2013 Nov 04; 1832(11):1906-9. PubMed ID: 23747979 [Abstract] [Full Text] [Related]
31. Adeno-associated virus 2-mediated gene therapy decreases autofluorescent storage material and increases brain mass in a murine model of infantile neuronal ceroid lipofuscinosis. Griffey M, Bible E, Vogler C, Levy B, Gupta P, Cooper J, Sands MS. Neurobiol Dis; 2004 Jul 04; 16(2):360-9. PubMed ID: 15193292 [Abstract] [Full Text] [Related]
32. Palmitoyl protein thioesterase 1 (PPT1) deficiency causes endocytic defects connected to abnormal saposin processing. Ahtiainen L, Luiro K, Kauppi M, Tyynelä J, Kopra O, Jalanko A. Exp Cell Res; 2006 May 15; 312(9):1540-53. PubMed ID: 16542649 [Abstract] [Full Text] [Related]
37. Production of lysophosphatidylcholine by cPLA2 in the brain of mice lacking PPT1 is a signal for phagocyte infiltration. Zhang Z, Lee YC, Kim SJ, Choi MS, Tsai PC, Saha A, Wei H, Xu Y, Xiao YJ, Zhang P, Heffer A, Mukherjee AB. Hum Mol Genet; 2007 Apr 01; 16(7):837-47. PubMed ID: 17341491 [Abstract] [Full Text] [Related]
38. A murine model of infantile neuronal ceroid lipofuscinosis-ultrastructural evaluation of storage in the central nervous system and viscera. Galvin N, Vogler C, Levy B, Kovacs A, Griffey M, Sands MS. Pediatr Dev Pathol; 2008 Apr 01; 11(3):185-92. PubMed ID: 17990914 [Abstract] [Full Text] [Related]