Related Experiment Video
Updated: Jan 23, 2026

Simultaneous Mapping and Quantitation of Ribonucleotides in Human Mitochondrial DNA
Published on: November 14, 2017
Biparental inheritance of mitochondrial DNA in humans is not a common phenomenon
Rocio Rius1,2, Mark J Cowley3,4,5, Lisa Riley6,7
1Murdoch Children's Research Institute, Melbourne, Australia.
Purpose:
A recent report has raised the possibility of biparental mitochondrial DNA (mtDNA) inheritance, which could lead to concerns by health-care professionals and patients regarding investigations and genetic counseling of families with pathogenic mitochondrial DNA variants. Our aim was to examine the frequency of this phenomenon by investigating a cohort of patients with suspected mitochondrial disease.
Methods:
We studied genome sequencing (GS) data of DNA extracted from blood samples of 41 pediatric patients with suspected mitochondrial disease and their parents.
Results:
All of the mtDNA variants in the probands segregated with their mother or were apparently de novo. There were no variants that segregated only with the father and none of these families showed evidence of biparental inheritance of their mtDNA.
Conclusion:
Paternal mitochondrial transmission is unlikely to be a common occurrence and therefore at this point we would not recommend changes in clinical practice.
Related Concept Videos
Genomic Imprinting and Inheritance
The expression of some genes depends on which parent passed the gene to the offspring, through a phenomenon known as...
Inheritance of Chromatin Structures
Animal Mitochondrial Genetics
Non-nuclear Inheritance
Export of Mitochondrial and Chloroplast Genes
Tip-of-the-Tongue Phenomenon

