Mitochondrial respiratory chain Complex I defects in Fanconi anemia complementation group A.
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Summary
An analysis of the respiratory fluxes in FANCA primary fibroblasts, lymphocytes and lymphoblasts and electron microscopy measures indeed show profound alterations in mitochondrial ultrastructure and shape, consistent with the altered morphology of FAN CA mitochondria.
- Type
- article
- Published
- 2013-10-01
- Cited by
- 65
- References
- 67
- OpenAlex
- https://openalex.org/W2024332756
- Semantic Scholar
- https://api.semanticscholar.org/CorpusID:237071
Keywords
FANCA, Fanconi anemia, Respiratory chain, Biology, Mitochondrial disease
References
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- Evidence of mitochondrial dysfunction and impaired ROS detoxifying machinery in Fanconi Anemia cells
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Cited by
- Dysregulated Ca2+ Homeostasis in Fanconi anemia cells
- Fanconi anemia (FA) and crosslinker sensitivity: Re‐appraising the origins of FA definition
- Inhibition of Metalloproteinase Activity in FANCA Is Linked to Altered Oxygen Metabolism
- Altered Calcium and Red-ox homeostasis underline defective haematopoiesis in Fanconi Anemia.
- Fasting induces anti-Warburg effect that increases respiration but reduces ATP-synthesis to promote apoptosis in colon cancer models
- Oxidative Stress and Mitochondrial Dysfunction across Broad-Ranging Pathologies: Toward Mitochondria-Targeted Clinical Strategies
- Mitochondrial respiratory complex I defects in Fanconi anemia.
- Treatment of FANCA Cells with Resveratrol and N-Acetylcysteine: A Comparative Study
- Mitochondrial dysfunction: a neglected component of skin diseases
- Changes in vimentin, lamin A/C and mitofilin induce aberrant cell organization in fibroblasts from Fanconi anemia complementation group A (FA-A) patients.
- High-Risk Human Papillomavirus E6 Protein Promotes Reprogramming of Fanconi Anemia Patient Cells through Repression of p53 but Does Not Allow for Sustained Growth of Induced Pluripotent Stem Cells
- Damaged mitochondria in Fanconi anemia – an isolated event or a general phenomenon?
- Update of the human and mouse Fanconi anemia genes
- Impairment of extramitochondrial oxidative phosphorylation in mouse rod outer segments by blue light irradiation.
- Discovery of a novel glucose metabolism in cancer: The role of endoplasmic reticulum beyond glycolysis and pentose phosphate shunt
- Evaluation of energy metabolism and calcium homeostasis in cells affected by Shwachman-Diamond syndrome
- AMP-activated protein kinase is involved in the activation of the Fanconi anemia/BRCA pathway in response to DNA interstrand crosslinks
- Why is an energy metabolic defect the common outcome in BMFS?
- Fancd2 in vivo interaction network reveals a non-canonical role in mitochondrial function
- Defects in mitochondrial energetic function compels Fanconi Anaemia cells to glycolytic metabolism.
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