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Journal Abstract Search

212 related items for PubMed ID: 21971706

  • 1. Altered glutamate receptor function in the cerebellum of the Ppt1-/- mouse, a murine model of infantile neuronal ceroid lipofuscinosis.
    Finn R, Kovács AD, Pearce DA.
    J Neurosci Res; 2012 Feb; 90(2):367-75. PubMed ID: 21971706
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  • 2. Cln3-mutations underlying juvenile neuronal ceroid lipofuscinosis cause significantly reduced levels of Palmitoyl-protein thioesterases-1 (Ppt1)-protein and Ppt1-enzyme activity in the lysosome.
    Appu AP, Bagh MB, Sadhukhan T, Mondal A, Casey S, Mukherjee AB.
    J Inherit Metab Dis; 2019 Sep; 42(5):944-954. PubMed ID: 31025705
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  • 6. Loss of Depalmitoylation Disrupts Homeostatic Plasticity of AMPARs in a Mouse Model of Infantile Neuronal Ceroid Lipofuscinosis.
    Koster KP, Flores-Barrera E, Artur de la Villarmois E, Caballero A, Tseng KY, Yoshii A.
    J Neurosci; 2023 Dec 06; 43(49):8317-8335. PubMed ID: 37884348
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  • 7. The novel Cln1(R151X) mouse model of infantile neuronal ceroid lipofuscinosis (INCL) for testing nonsense suppression therapy.
    Miller JN, Kovács AD, Pearce DA.
    Hum Mol Genet; 2015 Jan 01; 24(1):185-96. PubMed ID: 25205113
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  • 10. In a model of Batten disease, palmitoyl protein thioesterase-1 deficiency is associated with brown adipose tissue and thermoregulation abnormalities.
    Khaibullina A, Kenyon N, Guptill V, Quezado MM, Wang L, Koziol D, Wesley R, Moya PR, Zhang Z, Saha A, Mukherjee AB, Quezado ZM.
    PLoS One; 2012 Jan 01; 7(11):e48733. PubMed ID: 23139814
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  • 11. Ppt1-deficiency dysregulates lysosomal Ca++ homeostasis contributing to pathogenesis in a mouse model of CLN1 disease.
    Mondal A, Appu AP, Sadhukhan T, Bagh MB, Previde RM, Sadhukhan S, Stojilkovic S, Liu A, Mukherjee AB.
    J Inherit Metab Dis; 2022 May 01; 45(3):635-656. PubMed ID: 35150145
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  • 12. CNS-directed AAV2-mediated gene therapy ameliorates functional deficits in a murine model of infantile neuronal ceroid lipofuscinosis.
    Griffey MA, Wozniak D, Wong M, Bible E, Johnson K, Rothman SM, Wentz AE, Cooper JD, Sands MS.
    Mol Ther; 2006 Mar 01; 13(3):538-47. PubMed ID: 16364693
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  • 14. Disruption of adaptive energy metabolism and elevated ribosomal p-S6K1 levels contribute to INCL pathogenesis: partial rescue by resveratrol.
    Wei H, Zhang Z, Saha A, Peng S, Chandra G, Quezado Z, Mukherjee AB.
    Hum Mol Genet; 2011 Mar 15; 20(6):1111-21. PubMed ID: 21224254
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  • 15. The role of attenuated astrocyte activation in infantile neuronal ceroid lipofuscinosis.
    Macauley SL, Pekny M, Sands MS.
    J Neurosci; 2011 Oct 26; 31(43):15575-85. PubMed ID: 22031903
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  • 16. Developmental NMDA receptor dysregulation in the infantile neuronal ceroid lipofuscinosis mouse model.
    Koster KP, Francesconi W, Berton F, Alahmadi S, Srinivas R, Yoshii A.
    Elife; 2019 Apr 04; 8():. PubMed ID: 30946007
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  • 17. Cerebellar pathology and motor deficits in the palmitoyl protein thioesterase 1-deficient mouse.
    Macauley SL, Wozniak DF, Kielar C, Tan Y, Cooper JD, Sands MS.
    Exp Neurol; 2009 May 04; 217(1):124-35. PubMed ID: 19416667
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  • 18. The blood-brain barrier is disrupted in a mouse model of infantile neuronal ceroid lipofuscinosis: amelioration by resveratrol.
    Saha A, Sarkar C, Singh SP, Zhang Z, Munasinghe J, Peng S, Chandra G, Kong E, Mukherjee AB.
    Hum Mol Genet; 2012 May 15; 21(10):2233-44. PubMed ID: 22331300
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  • 19. Evaluation of neurodegeneration in a mouse model of infantile batten disease by magnetic resonance imaging and magnetic resonance spectroscopy.
    Munasinghe J, Zhang Z, Kong E, Heffer A, Mukherjee AB.
    Neurodegener Dis; 2012 May 15; 9(4):159-69. PubMed ID: 22327870
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  • 20. Deficiency of the INCL protein Ppt1 results in changes in ectopic F1-ATP synthase and altered cholesterol metabolism.
    Lyly A, Marjavaara SK, Kyttälä A, Uusi-Rauva K, Luiro K, Kopra O, Martinez LO, Tanhuanpää K, Kalkkinen N, Suomalainen A, Jauhiainen M, Jalanko A.
    Hum Mol Genet; 2008 May 15; 17(10):1406-17. PubMed ID: 18245779
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