يعرض 1 - 10 نتائج من 43 نتيجة بحث عن '"LYSOSOMAL storage diseases"', وقت الاستعلام: 0.85s تنقيح النتائج
  1. 1
    دورية أكاديمية
    Modeling neuronopathic storage diseases with patient-derived culture systems.

    المؤلفون: Zunke, Friederike1 (AUTHOR) fzunke@biochem.uni-kiel.de, Mazzulli, Joseph R.1 (AUTHOR) jmazzulli@northwestern.edu

    المصدر: Neurobiology of Disease. Jul2019, Vol. 127, p147-162. 16p.

    مصطلحات موضوعية: *INDUCED pluripotent stem cells, *LYSOSOMAL storage diseases, *METABOLIC disorders, *PATHOLOGY, *SOMATIC cells, *NEUROGLIA


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  2. 2
    دورية أكاديمية
    Lysosomal dysfunction and early glial activation are involved in the pathogenesis of spinocerebellar ataxia type 21 caused by mutant transmembrane protein 240.

    المؤلفون: Seki, Takahiro1 takaseki@kumamoto-u.ac.jp, Sato, Masahiro1, Kibe, Yuki1, Ohta, Tomoko1, Oshima, Mutsumi1, Konno, Ayumu1, Hirai, Hirokazu1, Kurauchi, Yuki1, Hisatsune, Akinori1, Katsuki, Hiroshi1

    المصدر: Neurobiology of Disease. Dec2018, Vol. 120, p34-50. 17p.

    مصطلحات موضوعية: *LYSOSOMAL storage diseases, *SPINOCEREBELLAR ataxia, *MEMBRANE proteins, *GENETIC mutation, *LABORATORY mice, *THERAPEUTICS


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  3. 3
    دورية أكاديمية
    Ellagic acid and its metabolites urolithins A/B ameliorate most common disease phenotypes in cellular and mouse models for lysosomal storage disorders by enhancing extracellular vesicle secretion.

    المؤلفون: Soto-Huelin, Beatriz1 (AUTHOR), Babiy, Bohdan2 (AUTHOR), Pastor, Oscar2 (AUTHOR), Díaz-García, Mario1 (AUTHOR), Toledano-Zaragoza, Ana1 (AUTHOR), Frutos, María Dolores3 (AUTHOR), Espín, Juan Carlos3 (AUTHOR), Tomás-Barberán, Francisco A.3 (AUTHOR), Busto, Rebeca1,4 (AUTHOR) rebeca.busto@hrc.es, Ledesma, María Dolores1 (AUTHOR) dledesma@cbm.csic.es

    المصدر: Neurobiology of Disease. Jun2023, Vol. 182, pN.PAG-N.PAG. 1p.

    مصطلحات موضوعية: *ELLAGIC acid, *LYSOSOMAL storage diseases, *EXTRACELLULAR vesicles, *GLYCOGEN storage disease type II, *LABORATORY mice, *METABOLITES

    النص الكامل

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  4. 4
    دورية أكاديمية
    Age-dependent alterations in neuronal activity in the hippocampus and visual cortex in a mouse model of Juvenile Neuronal Ceroid Lipofuscinosis (CLN3).

    المؤلفون: Burkovetskaya, Maria1, Karpuk, Nikolay1, Kielian, Tammy1 tkielian@unmc.edu

    المصدر: Neurobiology of Disease. Apr2017, Vol. 100, p19-29. 11p.

    مصطلحات موضوعية: *LYSOSOMAL storage diseases, *NEURAL circuitry, *HIPPOCAMPUS (Brain), *VISUAL cortex, *ELECTROPHYSIOLOGY, *LABORATORY mice, *DIAGNOSIS


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  5. 5
    دورية أكاديمية
    Lysosomal enzyme cathepsin B enhances the aggregate forming activity of exogenous α-synuclein fibrils.

    المؤلفون: Tsujimura, Atsushi1, Taguchi, Katsutoshi1, Watanabe, Yoshihisa1, Tatebe, Harutsugu2, Tokuda, Takahiko2, Mizuno, Toshiki2, Tanaka, Masaki1 mtanaka@koto.kpu-m.ac.jp

    المصدر: Neurobiology of Disease. Jan2015, Vol. 73, p244-253. 10p.

    مصطلحات موضوعية: *LYSOSOMAL storage diseases, *CATHEPSIN B, *ALPHA-synuclein, *PARKINSON'S disease, *LEWY body dementia, *FLUORESCENT proteins, *CELL imaging, *IMMUNOCYTOCHEMISTRY


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  6. 6
    دورية أكاديمية
    Sodium phenylbutyrate reverses lysosomal dysfunction and decreases amyloid-β42 in an in vitro-model of inclusion-body myositis.

    المؤلفون: Nogalska, Anna1, D'Agostino, Carla1, Engel, W. King1, Askanas, Valerie1 askanas@usc.edu

    المصدر: Neurobiology of Disease. May2014, Vol. 65, p93-101. 9p.

    مصطلحات موضوعية: *LYSOSOMAL storage diseases, *AMYLOID, *IN vitro studies, *INCLUSION body myositis, *MUSCLE diseases, *SECRETASES


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  7. 7
    دورية أكاديمية
    Lysosomal dysfunction increases exosome-mediated alpha-synuclein release and transmission

    المؤلفون: Alvarez-Erviti, Lydia1 l.alvarez@medsch.ucl.ac.uk, Seow, Yiqi2, Schapira, Anthony H.1, Gardiner, Chris3, Sargent, Ian L.3, Wood, Matthew J.A.2, Cooper, J. Mark1

    المصدر: Neurobiology of Disease. Jun2011, Vol. 42 Issue 3, p360-367. 8p.

    مصطلحات موضوعية: *PARKINSON'S disease, *LYSOSOMAL storage diseases, *PROTEINS, *AMMONIUM chloride, *MACROLIDE antibiotics, *NEURONS, *NEURAL transmission


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  8. 8
    دورية أكاديمية
    A new large animal model of CLN5 neuronal ceroid lipofuscinosis in Borderdale sheep is caused by a nucleotide substitution at a consensus splice site (c.571+1G>>>A) leading to excision of exon 3

    المؤلفون: Frugier, Tony1, Mitchell, Nadia L.1, Tammen, Imke2, Houweling, Peter J.2, Arthur, Donald G.3, Kay, Graham W.1, van Diggelen, Otto P.4, Jolly, Robert D.5, Palmer, David N.1 palmerd@lincoln.ac.nz

    المصدر: Neurobiology of Disease. Feb2008, Vol. 29 Issue 2, p306-315. 10p.

    مصطلحات موضوعية: *NEURONAL ceroid-lipofuscinosis, *LYSOSOMAL storage diseases, *MESSENGER RNA, *JUVENILE diseases


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  9. 9
    دورية أكاديمية
    The therapeutic potential of neural stem/progenitor cells in murine globoid cell leukodystrophy is conditioned by macrophage/microglia activation

    المؤلفون: Pellegatta, Serena1, Tunici, Patrizia1, Poliani, Pietro Luigi1,2, Dolcetta, Diego3, Cajola, Laura1, Colombelli, Cristina1, Ciusani, Emilio1, Di Donato, Stefano1, Finocchiaro, Gaetano1 finocchiaro@istituto-besta.it

    المصدر: Neurobiology of Disease. Feb2006, Vol. 21 Issue 2, p314-323. 10p.

    مصطلحات موضوعية: *GLOBOID cell leukodystrophy, *LYSOSOMAL storage diseases, *NEURAL stem cells, *GREEN fluorescent protein, *MICROGLIA


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  10. 10
    دورية أكاديمية
    An Inducible Mouse Model of Late Onset Tay–Sachs Disease

    المؤلفون: Jeyakumar, Mylvaganam1, Smith, David1, Eliott-Smith, Elena1, Cortina-Borja, Mario2, Reinkensmeier, Gabriele1, Butters, Terry D.1, Lemm, Thorsten3, Sandhoff, Konrad3, Perry, V. Hugh4, Dwek, Raymond A.1, Platt, Frances M.1

    المصدر: Neurobiology of Disease. Aug2002, Vol. 10 Issue 3, p201. 10p.

    مصطلحات موضوعية: *LYSOSOMAL storage diseases, *MICE, *GANGLIOSIDOSES, *NEURODEGENERATION


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