PURPOSE This study was designed to assess the effect of mitochondrial DNA (mtDNA) mutation T8993C on cone structure in a family expressing neurogenic muscle weakness, ataxia, and retinitis pigmentosa (NARP) syndrome. METHODS Five family members were studied, using clinical examination, nerve conduction studies, perimetry, optical coherence tomography (OCT) measures of central retinal thicknes...

Mitochondria are highly dynamic organelles that continuously move, fuse and divide. Mitochondrial dynamics modulate overall mitochondrial morphology and are essential for the proper function, maintenance and transmission of mitochondria and mitochondrial DNA (mtDNA). We have investigated mitochondrial fusion in yeast cells with severe defects in oxidative phosphorylation (OXPHOS) due to removal...

15 Poulton J, Morten K, Brown G, Bindoff L. Are duplications of mitochondrial DNA characteristic of Kearns-Sayre syndrome? Human Molecular Genetics 1994; 3: 947-51. 16 Prezant TR, Agapian JV, Bohlman MC, et al. Mitochondrial ribosomal RNA mutation associated with both antibiotic-induced and non-syndromic deafness. Nature Genet 1993; 4: 289-94. 17 Fischel-Ghodsian N, Prezant TR, Bu X, Oztas S. M...

Bacteria can use mammalian hormones to modulate pathogenic processes that play essential roles in disease development. Actinobacillus pleuropneumoniae is an important porcine respiratory pathogen causing great economic losses in the pig industry globally. Stress is known to contribute to the outcome of A. pleuropneumoniae infection. To test whether A. pleuropneumoniae could respond to stress ho...

CMYK 305 Mitochondrial neurological disorders remain esoteric but fascinating to the clinician. Aspects of their biology illuminate the complexities of their clinical manifestations. The hallmark multiple organ dysfunction is traceable to failure of mitochondria, the ubiquitous organelles of energy generation. High energy cell populations that are post-mitotic and nondividing at birth, are espe...

Introduction. Edible oils containing > 60 % monoenoic acids (i. e. oleic acid), in particular olive oil and Canola oil have been shown to exhibit beneficial effects on lipid metabolism in human (LDL-cholesterol blood levels, triacylglycerol blood levels) within the last few years [1]. These findings are in contrast to earlier recommandations by nutrition experts who strongly favored high-linole...