Rapid Progression of Cardiomyopathy in Mitochondrial Diabetes

Momiyama, Yukihiko; Atsumi, Yoshihito; Ohsuzu, Fumitaka; Ui, Susumu; Morinaga, Shojirou; Matsuoka, Kempei; Kimura, Masashi

doi:10.1253/jcj.63.130

Cited by 23 publications

(11 citation statements)

References 19 publications

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“…Mitochondria play an important role in the development of NIDDM, which agrees with an age-related decline in the capacity for oxidative phosphorylation, and consequently contributes to its pathophysiology [5,6]. In several animal models, a relationship has been established between diabetes and some dysfunctions in mitochondrial oxidative events [7,8], suggesting the occurrence of bioenergetic alterations at the mitochondrial level [7,9].…”

Section: Introductionmentioning

confidence: 94%

Diabetes and mitochondrial oxidative stress: A study using heart mitochondria from the diabetic Goto-Kakizaki rat

Santos¹,

Palmeira²,

Seiça³

et al. 2003

Vascular Biochemistry

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Increasing evidence shows that the overproduction of reactive oxygen species, induced by diabetic hyperglycemia, contributes to the development of several cardiopathologies. The susceptibility of diabetic hearts to oxidative stress, induced in vitro by ADP-Fe 2+ in mitochondria, was studied in 12-month-old Goto-Kakizaki rats, a model of non-insulin dependent diabetes mellitus, and normal (non-diabetic) Wistar rats. In terms of lipid peroxidation the oxidative damage was evaluated on heart mitochondria by measuring both the O 2 consumption and the concentrations of thiobarbituric acid reactive substances. Diabetic rats display a more intense formation of thiobarbituric acid reactive substances and a higher O 2 consumption than nondiabetic rats. The oxidative damage, assessed by electron microscopy, was followed by an extensive effect on the volume of diabetic heart mitochondria, as compared with control heart mitochondria. An increase in the susceptibility of diabetic heart mitochondria to oxidative stress can be explained by reduced levels of endogenous antioxidants, so we proceeded in determining α-tocopherol, GSH and coenzyme Q content. Although no difference of α-tocopherol levels was found in diabetic rats as compared with control rat mitochondria, a significant reduction in GSH (21.5% reduction in diabetic rats) and coenzyme Q levels of diabetic rats was observed. The data suggest that a significant decrease of coenzyme Q 9 , a potent antioxidant involved in the elimination of mitochondria-generated reactive oxygen species, may be responsible for an increased susceptibility of diabetic heart mitochondria to oxidative damage. (Mol Cell Biochem 246: 163-170, 2003)

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Section: Introductionmentioning

confidence: 94%

Diabetes and mitochondrial oxidative stress: A study using heart mitochondria from the diabetic Goto-Kakizaki rat

Santos¹,

Palmeira²,

Seiça³

et al. 2003

Vascular Biochemistry

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“…Mitochondrial diabetes is a progressive disorder in terms of the insulin secretory capacity (34) and the cardiac function (35). Thiazolidinediones may increase the total plasma volume, which worsens the cardiac function (36,37).…”

Section: Discussionmentioning

confidence: 99%

Treatment of Mitochondrial Diabetes with a Peroxisome Proliferator-activated Receptor (PPAR)-gamma Agonist

et al. 2016

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The 3243 A>G mutation in mitochondrial DNA is the most common cause of monogenic diabetes mellitus in Japan. A 45-year-old woman with mitochondrial diabetes and significant insulin resistance presented with hypoadiponectinemia despite a normal amount of visceral fat. Three months of treatment with pioglitazone (PIO) improved her blood glucose profile and response to the 75-g oral glucose tolerance test. These changes were accompanied by the amelioration of her insulin resistance and the impairment of early-phase insulin secretion. Her serum adiponectin levels increased to the normal range. In this case of mitochondrial diabetes, PIO was effective for glycemic control.

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“…Previous reports have indicated that the clinical features of mitochondrial cardiomyopathy in patients carrying the A3243G mutation show substantial variability. Hiruta et al described a patient with the A3243G mutation who presented with hypertrophic cardiomyopathy,3 whereas Momiyama et al reported on a patient with the same mutation who had dilated hypertrophic cardiomyopathy 4…”

Section: Discussionmentioning

confidence: 99%

A case of mitochondrial cardiomyopathy with restrictive transmitral filling pattern

Otsui¹,

Inoue²,

Tamagawa³

et al. 2012

IMCRJ

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A 61-year-old diabetic woman with a mitochondrial A3243G mutation was hospitalized for evaluation of breathlessness, general fatigue, and leg edema. Chest radiography revealed cardiomegaly with massive pleural effusion. Serum lactate, pyruvate, and brain natriuretic peptide concentrations were elevated. Transthoracic echocardiography revealed a restrictive pattern of transmitral flow, although systolic function of the left ventricle was only mildly impaired. Based on these findings and her clinical course, the patient was diagnosed with right-sided heart failure caused by mitochondrial cardiomyopathy associated with a restrictive transmitral filling pattern. Treatment with furosemide, enalapril, and eplerenone was effective, and improvement in her symptoms was associated with amelioration of transthoracic echocardiographic findings and a reduction in serum brain natriuretic peptide levels. Previous reports have indicated heterogeneity in the clinical features of mitochondrial cardiomyopathy in patients carrying the A3243G mutation; the present case highlights the substantial variability in the clinical features of this disease.

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Rapid Progression of Cardiomyopathy in Mitochondrial Diabetes

Cited by 23 publications

References 19 publications

Diabetes and mitochondrial oxidative stress: A study using heart mitochondria from the diabetic Goto-Kakizaki rat

Diabetes and mitochondrial oxidative stress: A study using heart mitochondria from the diabetic Goto-Kakizaki rat

Treatment of Mitochondrial Diabetes with a Peroxisome Proliferator-activated Receptor (PPAR)-gamma Agonist

A case of mitochondrial cardiomyopathy with restrictive transmitral filling pattern

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