MELAS syndrome and cardiomyopathy: linking mitochondrial function to heart failure pathogenesis.
Heart failure remains an important clinical burden, and mitochondrial dysfunction plays a key role in its pathogenesis. The heart has a high metabolic demand, and mitochondrial function is a key determinant of myocardial performance. In mitochondrial disorders, hypertrophic remodeling is the early p...
| Publicado en: | Heart Failure Reviews Vol. 21; no. 1; pp. 103 - 117 |
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| Autores principales: | , , , , , , , |
| Formato: | research Journal Article |
| Publicado: |
Springer Nature
Jan2016
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=112262483&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 112262483 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 13824147 OG1 jtl: Heart Failure Reviews issn: 13824147 maglogo: N pubinfo: dt: Jan2016 vid: 21 iid: 1 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 112262483 112262483 NLM26712328 112262483 10.1007/s10741-015-9524-5 NLM26712328 112262483 ppf: 103 ppct: 14 formats: tig: atl: MELAS syndrome and cardiomyopathy: linking mitochondrial function to heart failure pathogenesis. aug: au: Hsu, Ying-Han Yogasundaram, Haran Parajuli, Nirmal Valtuille, Lucas Sergi, Consolato Oudit, Gavin Hsu, Ying-Han R Oudit, Gavin Y affil: Department of Laboratory Medicine and Pathology, Mazankowski Alberta Heart Institute, University of Alberta, Edmonton Canada sug: subj: Mitochondrial Encephalomyopathies Physiopathology Myocardial Diseases Physiopathology Heart Failure Metabolism Mitochondria Metabolism Mitochondrial Encephalomyopathies Metabolism Myocardial Diseases Metabolism Myocardial Diseases Complications Mitochondrial Encephalomyopathies Complications Heart Failure Etiology Heart Failure Physiopathology Human ab: Heart failure remains an important clinical burden, and mitochondrial dysfunction plays a key role in its pathogenesis. The heart has a high metabolic demand, and mitochondrial function is a key determinant of myocardial performance. In mitochondrial disorders, hypertrophic remodeling is the early pattern of cardiomyopathy with progression to dilated cardiomyopathy, conduction defects and ventricular pre-excitation occurring in a significant proportion of patients. Cardiac dysfunction occurs in approximately a third of patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome, a stereotypical example of a mitochondrial disorder leading to a cardiomyopathy. We performed unique comparative ultrastructural and gene expression in a MELAS heart compared with non-failing controls. Our results showed a remarkable increase in mitochondrial inclusions and increased abnormal mitochondria in MELAS cardiomyopathy coupled with variable sarcomere thickening, heterogeneous distribution of affected cardiomyocytes and a greater elevation in the expression of disease markers. Investigation and management of patients with mitochondrial cardiomyopathy should follow the well-described contemporary heart failure clinical practice guidelines and include an important role of medical and device therapies. Directed metabolic therapy is lacking, but current research strategies are dedicated toward improving mitochondrial function in patients with mitochondrial disorders. pubtype: Academic Journal doctype: research Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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