Disease relevance of Cardiomyopathies

• A mouse model for mitochondrial myopathy and cardiomyopathy resulting from a deficiency in the heart/muscle isoform of the adenine nucleotide translocator [1].
• We suggest that PPAR-delta is a potential therapeutic target in treating lipotoxic cardiomyopathy and other heart diseases [2].
• Limb-girdle muscular dystrophy type 2E (LGMD 2E) is caused by mutations in the beta-sarcoglycan gene, which is expressed in skeletal, cardiac, and smooth muscle. beta-sarcoglycan-deficient (Sgcb-null) mice developed severe muscular dystrophy and cardiomyopathy with focal areas of necrosis [3].
• Letter: Pericarditis in a case of early daunorubicin cardiomyopathy [4].
• X-linked cardiomyopathy (XLCM) is a rapidly progressive primary myocardial disorder presenting in teenage males as congestive heart failure [5].

Psychiatry related information on Cardiomyopathies

• It is concluded that species- and tissue-specific differences in the distribution of antioxidant defense mechanisms may be primary determinants of the relative insensitivity of liver and, in contrast, the rather selective cardiomyopathy resulting from adriamycin administration in vivo [6].
• Stress-induced cardiomyopathy is described as an acute cardiomyopathy that occurs under the influence of an excessive level of catecholamine related to intense emotional stress [7].

High impact information on Cardiomyopathies

• Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits [8].
• Diagnosis of chloroquine cardiomyopathy by endomyocardial biopsy [9].
• Echocardiogram for amyloid cardiomyopathy [10].
• Thus, chronic myocardial PPAR-delta deficiency leads to lipotoxic cardiomyopathy [2].
• Adult mice deficient in actinin-associated LIM-domain protein reveal a developmental pathway for right ventricular cardiomyopathy [11].

Chemical compound and disease context of Cardiomyopathies

• Acute myocardiopathy following tripathway exposure to methylenedianiline [12].
• For elucidation of this question, the temporal relationship between the onset of doxorubicin-induced cardiomyopathy and nephropathy was studied [13].
• Interruption of verapamil treatment leads again to vascular dysfunction and acute myocardial necrosis, indicating that predilection for cardiomyopathy is a continuing process [14].
• To test the hypothesis that mismatch between myocardial fatty acid uptake and utilization leads to the accumulation of cardiotoxic lipid species, and to establish a mouse model of metabolic cardiomyopathy, we generated transgenic mouse lines that overexpress long-chain acyl-CoA synthetase in the heart (MHC-ACS) [15].
• Letter: Cobalt intoxication in uraemic myocardiopathy [16]?
• Overall, HRQL was not affected by ICD implantation in patients in the Defibrillators in Nonischemic Cardiomyopathy Treatment Evaluation study [17].

Biological context of Cardiomyopathies

• Since free radicals and lipid peroxidation are suggested to be involved in ADR cardiomyopathy, we examined the beneficial effects of probucol, a lipid-lowering drug with strong antioxidant properties [18].
• Although the basic functional and structural roles of cTnT in modulating contractility are relatively well understood, the mechanisms that link point mutations in cTnT to the development of this complex cardiomyopathy are unknown [19].
• Moreover, these data suggest that modulation of PI 3-kinase activities may represent a potential therapeutic strategy to counteract the occurrence of apoptosis in cardiomyopathy [20].
• IGF-1 overexpression inhibits the development of diabetic cardiomyopathy and angiotensin II-mediated oxidative stress [21].
• The development of diabetic cardiomyopathy might have involved downregulation of Hsp60 and subsequent reduction of IGF-1 receptor signaling [22].

Anatomical context of Cardiomyopathies

• Clinical and animal studies of morphological changes during the early stages of doxorubicin-induced cardiomyopathy have suggested that the sarcoplasmic reticulum, the intracellular membrane system responsible for myoplasmic calcium regulation in adult mammalian heart, may be the early target of doxorubicin [23].
• Function of myocardial mitochondria in the adriamycin-induced cardiomyopathy of rabbits [24].
• Limb-girdle muscular dystrophy types 2E and F are characterized by skeletal muscle weakness and often cardiomyopathy and are due to mutations in the genes encoding beta- and delta-sarcoglycan [25].
• Mice null for desmin, the muscle-specific member of the intermediate filament gene family, develop cardiomyopathy characterized by extensive cardiomyocyte death, fibrosis, calcification, and eventual heart failure [26].
• We suggest that a gene-related blunted coronary reserve resulting from an impairment of vascular smooth muscle is an early component of DM cardiomyopathy [27].

Gene context of Cardiomyopathies

• ACTC is the first sarcomeric gene described in which mutations are responsible for 2 different cardiomyopathies [28].
• METHODS AND RESULTS: Northern blot analyses were performed with probes to TIMP-1 to -4 and GAPDH with poly A+ mRNA from ventricular tissues of patients with ischemic cardiomyopathy (ICM, n=16) or idiopathic dilated cardiomyopathy (DCM, n=15) and nonfailing control hearts (n=15) [29].
• In addition, these findings suggest that caveolin-3 transgenic mice may represent a valid mouse model for studying the molecular mechanisms underlying cardiomyopathies associated with Duchenne muscular dystrophy [30].
• Here, we used mice with tissue-specific knockout of mitochondrial transcription factor A (Tfam) that leads to progressive cardiomyopathy [31].
• In the absence of genes under the control of Irx4, ventricular function deteriorates and cardiomyopathy ensues [32].

Analytical, diagnostic and therapeutic context of Cardiomyopathies

• Signal transducer and activator of transcription 3 in the heart transduces not only a hypertrophic signal but a protective signal against doxorubicin-induced cardiomyopathy [33].
• As in many human cardiomyopathies, transgenic mice developed spontaneous arrhythmias in vivo, and programmed electrical stimulation of isolated-perfused transgenic hearts demonstrated a strikingly high incidence of spontaneous and inducible ventricular tachycardia [34].
• Since complete transduction of the entire heart is an impractical goal, it becomes critical to determine the minimal level of correction needed for successful DMD cardiomyopathy gene therapy [35].
• A cardiomyopathy similar to that observed in cancer patients after prolonged chemotherapy with adriamycin can be produced in the rabbit, characterized by mitochondrial and myofilamentous degeneration with noninflammatory myolysis and connective tissue repair [36].
• Adriamycin-induced cardiomyopathy in rabbits was produced by intravenous injections of the drug with a short therapeutic schedule (3 mg/kg body wt administered as four intermittent doses) [37].

References