Cytochrome c Oxidase-deficient Patients Have Distinct Subunit Assembly Profiles

Hanson, Bonnie J.; Carrozzo, Rosalba; Piemonte, Fiorella; Tessa, Alessandra; Robinson, Brian H.; Capaldi, Roderick A.

doi:10.1074/jbc.m011162200

Cited by 37 publications

(25 citation statements)

References 47 publications

(55 reference statements)

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“…Due to its abundance, porin is often used as a reference standard in Western blot when assaying other mitochondrial proteins. 22 At the same time, another mitochondrial marker …”

Section: Resultsmentioning

confidence: 99%

Functional characterization of mitochondria in neutrophils: a role restricted to apoptosis

et al. 2003

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Mitochondria are known to combine life-supporting functions with participation in apoptosis by controlling caspase activity. Here, we report that in human blood neutrophils the mitochondria are different, because they preserve mainly death-mediating abilities. Neutrophil mitochondria hardly participate in ATP synthesis, and have a very low activity of the tested marker enzymes. The presence of mitochondria in neutrophils was confirmed by quantification of mitochondrial DNA copy number, by detection of mitochondrial porin, and by JC-1 measurement of Dw m . During neutrophilic differentiation, HL-60 cells demonstrated a profound cytochrome c depletion and mitochondrial shape change reminiscent of neutrophils. However, blood neutrophils containing extremely low amounts of cytochrome c displayed strong caspase-9 activation during apoptosis, which was also observed in apoptotic neutrophil-derived cytoplasts lacking any detectable cytochrome c. We suggest that other proapoptotic factors such as Smac/DIABLO and HtrA2/Omi, which are massively released from the mitochondria, have an important role in neutrophil apoptosis.

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“…Due to its abundance, porin is often used as a reference standard in Western blot when assaying other mitochondrial proteins. 22 At the same time, another mitochondrial marker …”

Section: Resultsmentioning

confidence: 99%

Functional characterization of mitochondria in neutrophils: a role restricted to apoptosis

et al. 2003

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“…We also assessed levels of porin, the most abundant protein of the mitochondrial outer membrane. Due to its abundance, porin is often used as a marker for cellular mitochondrial mass (17). Anti-␤-actin (no.…”

Section: ϫ4mentioning

confidence: 99%

Resveratrol induces mitochondrial biogenesis in endothelial cells

Csiszár

Labinskyy

Pinto

et al. 2009

American Journal of Physiology-Heart and Circulatory Physiology

382

300

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Pathways that regulate mitochondrial biogenesis are potential therapeutic targets for the amelioration of endothelial dysfunction and vascular disease. Resveratrol was shown to impact mitochondrial function in skeletal muscle and the liver, but its role in mitochondrial biogenesis in endothelial cells remains poorly defined. The present study determined whether resveratrol induces mitochondrial biogenesis in cultured human coronary arterial endothelial cells (CAECs). In CAECs resveratrol increased mitochondrial mass and mitochondrial DNA content, upregulated protein expression of electron transport chain constituents, and induced mitochondrial biogenesis factors (proliferator-activated receptor-coactivator-1α, nuclear respiratory factor-1, mitochondrial transcription factor A). Sirtuin 1 (SIRT1) was induced, and endothelial nitric oxide (NO) synthase (eNOS) was upregulated in a SIRT1-dependent manner. Knockdown of SIRT1 (small interfering RNA) or inhibition of NO synthesis prevented resveratrol-induced mitochondrial biogenesis. In aortas of type 2 diabetic (db/db) mice impaired mitochondrial biogenesis was normalized by chronic resveratrol treatment, showing the in vivo relevance of our findings. Resveratrol increases mitochondrial content in endothelial cells via activating SIRT1. We propose that SIRT1, via a pathway that involves the upregulation of eNOS, induces mitochondrial biogenesis. Resveratrol induced mitochondrial biogenesis in the aortas of type 2 diabetic mice, suggesting the potential for new treatment approaches targeting endothelial mitochondria in metabolic diseases.

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“…Patients 3 and 4 were from the Hospital for Sick Children (Toronto, Ont, Canada). These two patients have been described previously by Hanson et al (2001). Patients 5 and 7 were from Columbia University College of Physicians and Surgeons (New York, NY).…”

Section: Cell Linesmentioning

confidence: 99%

An Immunocytochemical Approach to Detection of Mitochondrial Disorders

Hanson

Capaldi

Marusich

et al. 2002

J Histochem Cytochem.

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S U M M A R YMitochondrial disorders can lead to a confusing array of symptoms, which frequently makes a diagnosis difficult. Traditional approaches to such diagnoses are based on enzyme activity assays, with further characterization provided by genetic analysis. However, these methods require relatively large sample sizes, are time-consuming, labor-intensive, and show variability between laboratories. Here, we report an immunocytochemical test that makes use of monoclonal antibodies to subunits from each of the oxidative phosphorylation complexes and pyruvate dehydrogenase to aid in the detection of mitochondrial disorders. It can be completed and data analyzed in less than 4 hr. We have used this test to study fibroblast cultures from patients with mitochondrial disorders arising from both mitochondrial DNA and nuclear DNA defects. We have also examined cases of Leigh syndrome arising from different genetic causes. We show that patients can be categorized on the basis of which complexes are affected and whether or not the defect being studied shows a mosaic distribution, an indicator of whether the causal mutation(s) is/are in the mitochondrial or nuclear genome. Immunocytochemical analysis as described here should be considered as an initial screen for mitochondrial disorders by which to direct (and limit) the subsequent enzymatic and genetic tests required to make an unambiguous diagnosis.

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Cytochrome c Oxidase-deficient Patients Have Distinct Subunit Assembly Profiles

Cited by 37 publications

References 47 publications

Functional characterization of mitochondria in neutrophils: a role restricted to apoptosis

Functional characterization of mitochondria in neutrophils: a role restricted to apoptosis

Resveratrol induces mitochondrial biogenesis in endothelial cells

An Immunocytochemical Approach to Detection of Mitochondrial Disorders

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