Send to

Choose Destination
Biochem Biophys Res Commun. 1994 Jul 15;202(1):102-10.

Fragmentation of human heart mitochondrial DNA associated with premature aging.

Author information

Department of Biomedical Chemistry, Faculty of Medicine, University of Nagoya, Japan.


Point mutations, oxygen damage and deletions in the heart mitochondrial (mt) DNA of a 19-year-old male patient with premature aging, who died of mitochondrial cardiomyopathy, were comprehensively analyzed. With total base-sequencing, one syn- mutation in the tRNA(Asp) gene and one mit-mutation in the ND3 gene were demonstrated. Using microHPLC/MS, 0.20% of the total deoxyguanosine (dG) were proved to be converted into its hydroxy-radical adduct, 8-hydroxy-dG, of which amount corresponds to that in normal subjects of 78 years old. The total detection system for mtDNA deletions, using 180 kinds of primer pairs, revealed extensive fragmentation of mtDNA; 235 types of deletions existed with various sizes, 97 of which yielded mtDNA minicircles lacking both of the replication origins of light- and heavy-strands. Deleted mtDNA accounted for 84% of the total mtDNA. In a man died from an accident at age 28 having almost the same mtDNA genotype except syn-, 50 types of deleted mtDNA, accounting for 15% of the total, were detected in his heart mtDNA. These results will present a clue to an unidentified mechanism of somatic mtDNA replication and the molecular basis of aging heart.

[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center