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|Title:||Fatal familial insomnia: mitochondrial and protein synthesis machinery decline in the mediodorsal thalamus|
|Author:||Frau Mendez, Margalida|
Blanco Tech, Rosa
Carmona Murillo, Margarita
Río Fernández, José Antonio del
Llorens Torres, Franc
Zarranz, Juan J.
Ferrer, Isidro (Ferrer Abizanda)
|Abstract:||The expression of subunits of mitochondrial respiratory complexes and components of the protein synthesis machinery from the nucleolus to the ribosome was analyzed in the mediodorsal thalamus in seven cases of Fatal Familial Insomnia (FFI) compared with age-matched controls. NDUFB8 (complex I subunit), SDHB (complex II subunit), UQCRC2 (complex III subunit), COX2 (complex IV subunit) and ATP50 (complex V subunit) expression levels, as revealed by western blotting, were reduced in FFI. Voltage-dependent anion channel (VDAC) and ATP5H were also reduced due to the marked depopulation of neurons. In contrast, a marked increase in superoxide dismutase 2 (SOD2) was found in reactive astrocytes thus suggesting that astrocytes are key factors in oxidative stress responses. The histone-binding chaperones nucleolin and nucleoplasmin 3, and histone H3 di-methylated K9 were markedly reduced together with a decrease in the expression of protein transcription elongation factor eEF1A. These findings show severe impairment in the expression of crucial components of mitochondrial function and protein synthesis in parallel with neuron loss in mediodorsal thalamus at terminal stages of FFI. Therapeutic measures must be taken long before the appearance of clinical symptoms to prevent the devastating effects of FFI.|
|Note:||Versió postprint del document publicat a: https://doi.org/10.1111/bpa.12408|
|It is part of:||Brain Pathology, 2017, vol. 27, num. 1, p. 95-106|
|Appears in Collections:||Articles publicats en revistes (Patologia i Terapèutica Experimental)|
Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
Articles publicats en revistes (Biologia Cel·lular, Fisiologia i Immunologia)
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