Two related proteolipids and dolichol-linked oligosaccharides accumulate in motor neuron degeneration mice (mnd/mnd), a model for neuronal ceroid lipofuscinosis
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Two related proteolipids and dolichol-linked oligosaccharides accumulate in motor neuron degeneration mice (mnd/mnd), a model for neuronal ceroid lipofuscinosis.
In this study, we show that two biochemical markers of neuronal ceroid lipofuscinoses (NCLs) are present in a mutant mouse (mnd/mnd) that exhibits symptoms of the disease. Subunit c of the mitochondrial F1F0-ATP synthase, a proteolipid that accumulates in storage bodies of most forms of NCL and several animal models, is dramatically increased in mnd/mnd mouse brain, kidney, liver, heart, and pa...
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acid. Tryptic peptides were prepared from the oligosaccharide-containing CNBr peptide (see below) by incubation at 2 mg/ml in 2% (w/v) ammonium bicarbonate, pH 8.0, with trypsin (Worthington TRTPCK; E:S of I : 100) at 25°C for 3 h, followed by freeze-drying. The peptides were dissolved in 0.1% (v/v) trifluoracetic acid and separated on a 4.6 mmx250 mm C-18 reverse-phase column (Vydac 201TP 104;...
متن کاملRetinal degeneration in motor neuron degeneration: a mouse model of ceroid lipofuscinosis.
PURPOSE To evaluate the retinal degeneration of the motor neuron degeneration (mnd) mouse, and to confirm its inheritance pattern and gene location. METHODS In screening the mnd/mnd mouse for ocular disease, a retinal degeneration was found that was evaluated by serial electroretinography, histology, electron microscopy, indirect ophthalmoscopy, and genetic and linkage analysis. RESULTS In ...
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An 11-year-old girl with juvenile neuronal ceroid lipofuscinosis developed acute pancreatitis secondary to valproic acid treatment, which resolved after discontinuation of the medication and conservative treatment. To my knowledge, this is the first reported case of neuronal ceroid lipofuscinosis with valproate-associated pancreatitis. Children with neuronal ceroid lipofuscinosis may be suscept...
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The neuronal ceroid-lipofuscinoses are a group of diseases characterized by progressive accumulation of autofluorescent lipopigments within neurons with associated neuronal damage." Variations of these diseases occur which include accumulations of lipopigments in viscera. The diseases long have been recognized in man where earlier studies of the pathogenesis suggested an increased rate of perox...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 1994
ISSN: 0021-9258
DOI: 10.1016/s0021-9258(17)37002-3