The calcium abnormality in cystic fibrosis mitochondria: Relative role of respiration and ATP hydrolysis
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Cited by (19)
CFTR activity and mitochondrial function
2013, Redox BiologyCitation Excerpt :On the other hand, Congdon and Littlewood found a glucose 6-phosphate dehydrogenase (G6PD or G6PDH) deficiency in CF [124], although this observation was not further explored. During the decade before the cloning of CFTR, Shapiro and Feigal reported the existence of different mitochondrial abnormalities in CF [108–113,115,117,125]. First, in 1979, based on previous work from other laboratories [126–130], they found alterations on calcium uptake and oxygen consumption in mitochondria isolated from fibroblasts of CF patients [113].
CISD1 codifies a mitochondrial protein upregulated by the CFTR channel
2008, Biochemical and Biophysical Research CommunicationsCitation Excerpt :We have also shown recently that another gene, MTND4, which is essential for the assembly and activity of the mitochondrial Complex I, has also a reduced expression in CF cells [6]. These results are all in agreement with early observations of a possible mitochondrial failure in CF [13–22], an idea apparently left aside when the CFTR gene was cloned and found to be a chloride channel. Further studies are required to determine if the reduced expression of CISD1 actually affects the activity of the mitochondrial Complex I.
Assessment of total energy expenditure in free-living patients with cystic fibrosis
1991, The Journal of PediatricsEvidence for a mitochondrial lesion in cystic fibrosis
1989, Life Sciences