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Dysfunctions of Cellular Oxidative Metabolism in Patients with Mutations in the NDUFS1 and NDUFS4 Genes of Complex I*
TLDR
Exposure of the NDUFS1 mutant fibroblasts to dibutyryl-cAMP stimulated the residual NADH-ubiquinone oxidoreductase activity, induced disappearance of ROS, and restored the mitochondrial potential. Expand
Hepatitis C virus protein expression causes calcium‐mediated mitochondrial bioenergetic dysfunction and nitro‐oxidative stress
TLDR
It was shown that HCV protein expression has a profound effect on cell oxidative metabolism, with specific inhibition of complex I activity, depression of mitochondrial membrane potential and oxidative phosphorylation coupling efficiency, increased production of reactive oxygen and nitrogen species, as well as loss of the Pasteur effect. Expand
Mitochondrial Respiratory Dysfunction in Familiar Parkinsonism Associated with PINK1 Mutation
TLDR
Mitochondrial respiratory function of fibroblasts from a patient affected by early-onset Parkinsonism carrying the homozygous W437X nonsense mutation in the PINK1 gene has been thoroughly characterized and exhibited a lower respiratory activity and a decreased respiratory control ratio. Expand
Bone-marrow derived hematopoietic stem/progenitor cells express multiple isoforms of NADPH oxidase and produce constitutively reactive oxygen species.
TLDR
It is shown that human hematopoietic stem/progenitor cells (HSCs) constitutively generate low levels of hydrogen peroxide whose production is inhibited by DPI, apocynin, catalase, and LY294002 and scarcely stimulated by PMA. Expand
Hepatitis C Virus-Linked Mitochondrial Dysfunction Promotes Hypoxia-Inducible Factor 1α-Mediated Glycolytic Adaptation
TLDR
It is shown that HCV protein expression activates hypoxia-inducible factor 1 (HIF-1) by normoxic stabilization of its α subunit, providing new insights into the pathogenesis of chronic hepatitis C and, possibly, the HCV-related development of hepatocellular carcinoma. Expand
The hypoxia‐inducible factor is stabilized in circulating hematopoietic stem cells under normoxic conditions
TLDR
It is shown that HIF‐1α stabilization correlates with down‐regulation of the tumour suppressor von Hippel‐Lindau protein (pVHL) and is positively controlled by NADPH‐oxidase‐dependent production of reactive oxygen species, indicating a specific O2‐independent post‐transcriptional control of HIF in mobilized HSCs. Expand
Coexistence of mutations in PINK1 and mitochondrial DNA in early onset parkinsonism
TLDR
This is the first report showing co-segregation of a Parkinson’s disease related nuclear gene mutation with mtDNA mutation(s), highlighting the hitherto unappreciated impact of coexisting mtDNA mutations in determining the development and the clinical course of the disease. Expand
Insulin resistance, steatosis and hepatitis C virus
TLDR
HCV treatment, shown able to decrease both the occurrence of HCV-related IR and diabetes, may reduce the risk of the associated morbidities. Expand
HCV infection induces mitochondrial bioenergetic unbalance: causes and effects.
TLDR
A temporal sequence of events is proposed in the HCV-infected cell whereby the primary alteration consists of a release of Ca(2+) from the endoplasmic reticulum, followed by uptake into mitochondria, which causes successive mitochondrial alterations comprising generation of reactive oxygen and nitrogen species and impairment of the oxidative phosphorylation. Expand
Chronic pro-oxidative state and mitochondrial dysfunctions are more pronounced in fibroblasts from Down syndrome foeti with congenital heart defects.
TLDR
The data suggest that an altered bioenergetic background in trisomy 21 foeti might be among the factors responsible for a more severe phenotype, and since the mitochondrial functional alterations might be rescued following pharmacological treatments, these results are of interest in the light of potential therapeutic interventions. Expand
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