First mitochondrial transplant in six children shows promise

Subject: Science and Technology

Context:

In a world first, six children with a rare disorder caused by deletions in the genomes of their mitochondria– the cellular compartments essential for energy generation- have been successfully treated with donor mitochondria from their mother.

More on the news:

Doctors transferred healthy mitochondria into the children’s haematopoietic stem cells, which give rise to blood cells.

About Mitochondrial replacement therapy (MRT):

Mitochondrial replacement therapy (MRT), sometimes called mitochondrial donation, is the replacement of mitochondria in one or more cells to prevent or ameliorate disease.
MRT originated as a special form of in vitro fertilisation in which some or all of the future baby’s mitochondrial DNA (mtDNA) comes from a third party.
This technique is used in cases when mothers carry genes for mitochondrial diseases.
The therapy is approved for use in the United Kingdom.
A second application is to use autologous mitochondria to replace mitochondria in damaged tissue to restore the tissue to a functional state.
This has been used in clinical research in the United States to treat cardiac-compromised newborns.

What is Mitochondria?

It was first described by a German pathologist named Richard Altmann in the year 1890.
Popularly known as the “Powerhouse of the cell,”mitochondria are a double membrane-bound organelle found in most eukaryotic organisms.
They are found inside the cytoplasm and essentially function as the cell’s “digestive system.”
They play a major role in breaking down nutrients and generating energy-rich molecules for the cell.
Many of the biochemical reactions involved in cellular respiration take place within the mitochondria.

Functions of Mitochondria:

The most important function of mitochondria is to produce energy through the process of oxidative phosphorylation.
It is also involved in the following process:
- Regulates the metabolic activity of the cell
- Promotes the growth of new cells and cell multiplication
- Helps in detoxifying ammonia in the liver cells
- Plays an important role in apoptosis or programmed cell death
- Responsible for building certain parts of the blood and various hormones like testosterone and oestrogen
- Helps in maintaining an adequate concentration of calcium ions within the compartments of the cell
- It is also involved in various cellular activities like cellular differentiation, cell signalling, cell senescence, controlling the cell cycle and also in cell growth.
Mitochondrial diseases: Alpers Disease, Barth Syndrome, Kearns-Sayre syndrome (KSS).