Histochemical localization of palmitoyl protein thioesterase-1 activity
نویسندگان
چکیده
منابع مشابه
STUDIES ON PALMITOYL-PROTEIN THIOESTERASE 1 Implications for synaptic functions
PDF created with FinePrint pdfFactory Pro trial version enzyme implicated in neurodegeneration, is localized in neurons and is developmentally regulated in rat brain. The expression of palmitoyl-protein thioesterase is developmentally regulated in neural tissues but not in nonneural tissues. changes in the expression of neuronal ceroid lipofuscinoses-linked proteins. Mol Genet Status epilepticu...
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Mutations in the depalmitoylating enzyme gene, PPT1, cause the infantile form of Neuronal Ceroid Lipofuscinosis (NCL), an early onset neurodegenerative disease. During recent years there have been different therapeutic attempts including enzyme replacement. Here we show that PPT1 is palmitoylated in vivo and is a substrate for two palmitoylating enzymes, DHHC3 and DHHC7. The palmitoylated prote...
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Infantile neuronal ceroid lipofuscinosis (INCL, Infantile Batten Disease) is an inherited, neurodegenerative lysosomal storage disorder. INCL is the result of a CLN1 gene mutation leading to a deficiency in palmitoyl protein thioesterase 1 (PPT1) activity. Studies in the forebrain demonstrate the PPT1-deficient mouse (PPT1-/-) mimics the clinical symptoms and underlying pathology of INCL; howev...
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A high capacity for accumulation of Mn was reported for sunflower plants. Localization of excess Mn is therefore of special interest for understanding metal tolerance mechanisms in this species. In this study, structural and histochemical alterations caused by Mn accumulation in leaves were investigated in sunflower (Helianthus annuus L. cv. Azar-ghol) plants grown in nutrient solution. In the ...
متن کاملPalmitoyl-protein thioesterase-1 deficiency leads to the activation of caspase-9 and contributes to rapid neurodegeneration in INCL.
The infantile neuronal ceroid lipofuscinosis (INCL), a rare (one in 100 000 births) but one of the most lethal inherited neurodegenerative storage disorders of childhood, is caused by inactivating mutations in the palmitoyl-protein thioesterase-1 (PPT1) gene. PPT1 cleaves thioester linkages in s-acylated (palmitoylated) proteins and facilitates their degradation and/or recycling. Thus, PPT1-def...
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ژورنال
عنوان ژورنال: Molecular Genetics and Metabolism
سال: 2016
ISSN: 1096-7192
DOI: 10.1016/j.ymgme.2015.11.004