Decrease of mitochondrial protein levels in human OPMD muscle biopsies.
Aymeric Chartier
Pierre Klein
Stéphanie Pierson
Nicolas Barbezier
Teresa Gidaro
François Casas
Steven Carberry
Paul Dowling
Laurie Maynadier
Maëlle Bellec
Martine Oloko
Claude Jardel
Bodo Moritz
George Dickson
Vincent Mouly
Kay Ohlendieck
Gillian Butler-Browne
Capucine Trollet
Martine Simonelig
10.1371/journal.pgen.1005092.g008
https://plos.figshare.com/articles/figure/_Decrease_of_mitochondrial_protein_levels_in_human_OPMD_muscle_biopsies_/1359257
<p>A) Distribution of differentially expressed proteins from OPMD and control human muscle samples, following a quantitative label-free LC-MS profiling proteomic analysis. Proteins are sorted according to their GO terms in "cellular component" using DAVID. Each square represents a protein. The value of the fold change is color-coded. Yellow to red, over-expressed in OPMD samples (maximum 20-fold); blue, under-expressed in OPMD samples (minimum -20-fold). B) Number of subunits of the mitochondrial respiratory chain complexes down-regulated in OPMD patient muscle biopsies. C) Quantification of levels of mRNAs encoding mitochondrial respiratory chain subunits in control and OPMD patient muscle biopsies, using RT-qPCR. Normalization was with B2M (Beta-2 microglobulin) mRNA. Box plots are from 4–7 muscle biopsies, the median is indicated as an horizontal line within the box. * <i>p</i>-value <0.05 using the Student’s t-Test. For ATP5B and ATP5F1, the median is lower in OPMD than in control biopsies, although the difference is not recorded as significant. D) Quantification of mitochondrial DNA on muscle biopsies, using qPCR. The MT-RNR1 mitochondrial gene was quantified. Normalization was with B2M nuclear DNA. Means are from four biological replicates, error bars represent standard deviation. ns: not significant, using the Student’s t-Test.</p>
2015-03-27 04:22:30
ccr
mRNAs encoding mitochondrial proteins
cleavage
poly
Drosophila OPMD model uncovers
deadenylation regulator Smaug
pabpn
candidate genes encoding RNA binding proteins
role
Oculopharyngeal Muscular Dystrophy Pathogenesis Oculopharyngeal
mitochondrial dysfunction