Document Detail

Dysregulation of Cardiolipin Biosynthesis in Pediatric Heart Failure.
MedLine Citation:
PMID:  24937604     Owner:  NLM     Status:  Publisher    
BACKGROUND: Cardiolipin, a unique phospholipid in the inner mitochondrial membrane, is critical for optimal mitochondrial function. CL abnormalities have been demonstrated in the failing rodent and adult human heart. The aim of this study was to determine whether abnormalities in CL content and the CL biosynthesis and remodeling pathways are present in pediatric idiopathic dilated cardiomyopathy (IDC).
METHODS AND RESULTS: A cross-sectional analysis of myocardial tissue from 119 IDC and non-failing (NF) control samples was performed. Electrospray ionizing mass spectrometry was used to measure total CL and CL species content in LV tissue. RT-PCR was employed to measure gene expression of the enzymes in the CL biosynthesis and remodeling pathways in both the adult and pediatric heart. Significantly lower total and (18:2)4CL (the beneficial species) content was demonstrated in myocardium from pediatric patients with IDC compared to NF controls. Analysis of mitochondrial gene transcripts was used to demonstrate that there is no decrease in mitochondrial content. Expression of two biosynthesis enzymes and one remodeling enzyme was significantly lower in pediatric IDC compared to NF controls. Expression of two phospholipases involved in CL degradation were also altered, one up and one down-regulated. Except for one remodeling enzyme, these changes are unique from those in the failing adult heart.
CONCLUSION: Similar to what has been seen in adults and in a rat model of IDC, total and (18:2)4CL are lower in pediatric IDC. Unique CL species profiles are seen in heart tissue from children with IDC compared to adults. Differences in CL biosynthesis and remodeling enzyme expression likely explain the differences in CL profiles observed in IDC and implicate unique age-related mechanisms of disease.
Kathryn C Chatfield; Genevieve C Sparagna; Carmen C Sucharov; Shelley D Miyamoto; Jonathan E Grudis; Rebecca D Sobus; Jamie Hijmans; Brian L Stauffer
Related Documents :
978134 - Alternative pathway of complement: recruitment of precursor properdin by the labile c3/...
3769194 - Serum enzymes in acute myocardial infarction after intracoronary thrombolysis.
17363004 - Lipopolysaccharide alters nucleotidase activities from lymphocytes and serum of rats.
22014644 - The complexity of cardiolipin in health and disease.
80314 - Chromatographic and electrophoretic heterogeneity of the cytochromes p-450 solubilized ...
16348784 - Purification and characterization of a keratinase from a feather-degrading bacillus lic...
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-6-14
Journal Detail:
Title:  Journal of molecular and cellular cardiology     Volume:  -     ISSN:  1095-8584     ISO Abbreviation:  J. Mol. Cell. Cardiol.     Publication Date:  2014 Jun 
Date Detail:
Created Date:  2014-6-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0262322     Medline TA:  J Mol Cell Cardiol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2014. Published by Elsevier Ltd.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  High-mobility group box 1 (HMGB1) impaired cardiac excitation-contraction coupling by enhancing the ...
Next Document:  Hydrolytic enzymes conjugated to quantum dots mostly retain whole catalytic activity.