Impairment of mitochondrial respiratory chain enzyme activities in tetralogy of fallot

Shinde, Santosh; Save, Vipul; Patil, Neela; Mishra, Kaushala Prasad; Ag, Tendolkar

doi:10.1016/j.cca.2006.09.011

Cited by 13 publications

(7 citation statements)

References 26 publications

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“…However, prolonged challenges to the heart may cause abnormal mitochondrial activity and morphology. Several reports showed decreased mitochondrial enzyme activity in TOF hearts [12,13]. Our results also showed vacuolar degeneration and breakage of the mitochondrial cristae in the myocardium of TOF patients ( Figure 1A).…”

Section: Discussionsupporting

confidence: 69%

“…A large body of work has been established to suggest that congenital and acquired heart diseases are associated with mitochondrial abnormalities [9][10][11]. Reduced mitochondrial enzymatic activities [12,13] and mitochondrial abnormalities [14,15] have been reportedly found in the hearts of TOF patients. PGC-1α, a potent transcriptional coactivator of many nuclear receptors and transcription factors, serves as a major regulator of mitochondrial biogenesis and activity [16][17][18][19].…”

Section: Introductionmentioning

confidence: 99%

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Hypoxia induces PGC-1α expression and mitochondrial biogenesis in the myocardium of TOF patients

et al. 2010

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PGC-1α, a potent transcriptional coactivator, is the major regulator of mitochondrial biogenesis and activity in the cardiac muscle. The dysregulation of PGC-1α and its target genes has been reported to be associated with congenital and acquired heart diseases. By examining myocardium samples from patients with Tetralogy of Fallot, we show here that PGC-1α expression levels are markedly increased in patients compared with healthy controls and positively correlated with the severity of cyanosis. Furthermore, hypoxia significantly induced the expression of PGC-1α and mitochondrial biogenesis in cultured cardiac myocytes. Mechanistic studies suggest that hypoxia-induced PGC-1α expression is regulated through the AMPK signaling pathway. Together, our data indicate that hypoxia can stimulate the expression of PGC-1α and mitochondrial biogenesis in the cardiac myocytes, and this process might provide a potential adaptive mechanism for cardiac myocytes to increase ATP output and minimize hypoxic damage to the heart.

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Section: Discussionsupporting

confidence: 69%

Section: Introductionmentioning

confidence: 99%

Hypoxia induces PGC-1α expression and mitochondrial biogenesis in the myocardium of TOF patients

et al. 2010

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“…It is proposed that myocardial bioenergetic failure worsens the clinical outcome of human subjects undergoing heart surgery for valvular defects, and that the investigation of mitochondrial function in myocardial biopsies is desirable in order to predict the postoperative cardiac mechanical complications [77]. To date, the approach to the investigation of mitochondrial function varies between laboratories.…”

Section: Cardiac and Skeletal Muscle Mitochondrial Dysfunction In Hfmentioning

confidence: 99%

Mitochondrial dysfunction in heart failure

2012

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Heart failure (HF) is a complex chronic clinical syndrome. Energy deficit is considered to be a key contributor to the development of both cardiac and skeletal myopathy. In HF several components of cardiac and skeletal muscle bioenergetics are altered, such as oxygen availability, substrate oxidation, mitochondrial ATP production, and ATP transfer to the contractile apparatus via the creatine kinase shuttle. This review focuses on alterations in mitochondrial biogenesis and respirasome organization, substrate oxidation coupled with ATP synthesis in the context of their contribution to the chronic energy deficit, and mechanical dysfunction of the cardiac and skeletal muscle in HF. We conclude that HF is associated with decreased mitochondrial biogenesis and function in both heart and skeletal muscle, supporting the concept of a systemic mitochondrial cytopathy. The sites of mitochondrial defects are located within the electron transport and phosphorylation apparatus, and differ with the etiology and progression of HF in the two mitochondrial populations (subsarcolemmal and interfibrillar) of cardiac and skeletal muscle. The roles of adrenergic stimulation, the renin-angiotensin system, and cytokines are evaluated as factors responsible for the systemic energy deficit. We propose a cylic AMP-mediated mechanism by which increased adrenergic stimulation contributes to the mitochondrial dysfunction.

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“…These authors reported a strong positive correlation of antioxidant, metabolic and angiogenic gene expression with pre‐operative oxygen saturation, and an inverse correlation between pre‐operative oxygen saturation and collagen gene expression. Mitochondria isolated from tetralogy of Fallot right ventricular outflow muscle have been reported to exhibit reduced activity of respiratory chain protein complexes and reduced cytochrome content compared with post‐mortem controls 29 . Tetralogy of Fallot patients with a C242T allele polymorphism for the p22 subunit of NADPH oxidase exhibit markedly reduced levels of low molecular weight protein tyrosine kinase phosphatase and methemoglobin reductase, reduced systolic and diastolic blood pressure and significantly higher plasma erythropoietin compared with age‐matched controls 30 .…”

Section: Discussionmentioning

confidence: 99%

Increased myocardial methionine‐enkephalin with reduced arterial oxygenation in congenital heart disease

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Cochrane

Rosenfeldt

et al. 2014

J Paediatrics Child Health

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Impairment of mitochondrial respiratory chain enzyme activities in tetralogy of fallot

Cited by 13 publications

References 26 publications

Hypoxia induces PGC-1α expression and mitochondrial biogenesis in the myocardium of TOF patients

Hypoxia induces PGC-1α expression and mitochondrial biogenesis in the myocardium of TOF patients

Mitochondrial dysfunction in heart failure

Increased myocardial methionine‐enkephalin with reduced arterial oxygenation in congenital heart disease

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