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Journal Abstract Search

239 related items for PubMed ID: 24056696

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  • 2. Cln1-mutations suppress Rab7-RILP interaction and impair autophagy contributing to neuropathology in a mouse model of infantile neuronal ceroid lipofuscinosis.
    Sarkar C, Sadhukhan T, Bagh MB, Appu AP, Chandra G, Mondal A, Saha A, Mukherjee AB.
    J Inherit Metab Dis; 2020 Sep; 43(5):1082-1101. PubMed ID: 32279353
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  • 3. 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
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  • 4. Reduction of neuroinflammation and seizures in a mouse model of CLN1 batten disease using the small molecule enzyme mimetic, N-Tert-butyl hydroxylamine.
    Fyke Z, Johansson R, Scott AI, Wiley D, Chelsky D, Zak JD, Al Nakouzi N, Koster KP, Yoshii A.
    Mol Genet Metab; 2024 Oct 01; 143(1-2):108537. PubMed ID: 39033629
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  • 9. 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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  • 12. 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 15; 45(3):635-656. PubMed ID: 35150145
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  • 13. 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 15; 90(2):367-75. PubMed ID: 21971706
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  • 14. Palmitoyl-protein thioesterase-1 deficiency leads to the activation of caspase-9 and contributes to rapid neurodegeneration in INCL.
    Kim SJ, Zhang Z, Lee YC, Mukherjee AB.
    Hum Mol Genet; 2006 May 15; 15(10):1580-6. PubMed ID: 16571600
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  • 15. Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals, contributing to neuropathology in humans and mice.
    Kim SJ, Zhang Z, Sarkar C, Tsai PC, Lee YC, Dye L, Mukherjee AB.
    J Clin Invest; 2008 Sep 15; 118(9):3075-86. PubMed ID: 18704195
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  • 17. 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 Sep 15; 7(11):e48733. PubMed ID: 23139814
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  • 19. 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
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