کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
1930008 1536784 2011 6 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Involvement of the mitochondrial compartment in human NCL fibroblasts
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی زیست شیمی
پیش نمایش صفحه اول مقاله
Involvement of the mitochondrial compartment in human NCL fibroblasts
چکیده انگلیسی

Neuronal ceroid lipofuscinosis (NCL) are a group of progressive neurodegenerative disorders of childhood, characterized by the endo-lysosomal storage of autofluorescent material. Impaired mitochondrial function is often associated with neurodegeneration, possibly related to the apoptotic cascade. In this study we investigated the possible effects of lysosomal accumulation on the mitochondrial compartment in the fibroblasts of two NCL forms, CLN1 and CLN6. Fragmented mitochondrial reticulum was observed in all cells by using the intravital fluorescent marker Mitotracker, mainly in the perinuclear region. This was also associated with intense signal from the lysosomal markers Lysotracker and LAMP2. Likewise, mitochondria appeared to be reduced in number and shifted to the cell periphery by electron microscopy; moreover the mitochondrial markers VDCA and COX IV were reduced following quantitative Western blot analysis. Whilst there was no evidence of increased cell death under basal condition, we observed a significant increase in apoptotic nuclei following Staurosporine treatment in CLN1 cells only. In conclusion, the mitochondrial compartment is affected in NCL fibroblasts invitro, and CLN1 cells seem to be more vulnerable to the negative effects of stressed mitochondrial membrane than CLN6 cells.


► Mitochondrial reticulum fragmentation occurs in human CLN1 and CLN6 fibroblasts.
► Likewise mitochondrial shift-to periphery and decreased mitochondrial density are seen.
► Enhanced caspase-mediated apoptosis occurs following STS treatment in CLN1 fibroblasts.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochemical and Biophysical Research Communications - Volume 416, Issues 1–2, 9 December 2011, Pages 159–164
نویسندگان
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