The Mitochondria Story

The first UK baby created with DNA from three people has been born in May 2023 after doctors performed a groundbreaking IVF procedure. The baby received its 23 pairs of chromosome from both of its biological parents. but the mother has an unhealthy mitochondria. So a Healthy mitochondria from a third woman was implanted artificially in the laboratory. The baby had genetic material from 3 parents. This is the first 3-parent Baby of our world.

Why Mitochondria is as important as the nucleus ?

We are able to see because a protein in the retina inside our eye ( Rhodopsin) is bleached. This generates an electric signal. This impulse is conveyed to the occipital part of our brain where the image of the object is created. Only after that we can perceive that object. When we are talking, a battery of proteins in our muscles are flexing and contracting. The enzymes that digest our food and the Insulin that prevents diabetes are all proteins.

Our DNA that codes for these 20,000 odd genes that run the daily business of living, reside inside the nucleus of our cell. The foetus inside the womb inherits half of these codes from mother and the other half from its biological father.

But do all our genes reside inside the nucleus ? Apparently, not all.

Nucleus is NOT the sole location of all our genes ?

Outside Nucleus, our body has one more source of  Genetic material- the Mitochondria. These are also shared to us during our birth.

Our mitochondria has 37 genes. 

They are different from the regular 23 pairs of chromosomes that resides inside the Z plus security of our nucleus. These mitochondrial Genes are the cooking stoves of our body. They convert the food we take into packets of energy known as ATP. Our brain, heart and muscles are energy guzzling equipments. They can’t function for a second without a healthy mitochondria. 

Mitochondrion is the kitchen of our cells.

All energy giving food that we eat are chopped down inside our body into their smallest unit called glucose and are ferried deep into our cells. Inside our cells, there are special locations called the mitochondria. Glucose is burnt on the stoves inside the mitochondria and ATP is formed. ATP is the currency of energy. It is required for each and every action and reaction of our body.

Mitochondria is the kitchen of our cell. Any defect in their code seriously hampers our ability to generate energy from the food we take. This is incompatible with life.

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Mitochondria are alien.

Believe me, mitochondria are  a separate micro-organism. In the beginning, this world was full of only simple, single cellular organisms. These cells digested food, converted them into energy and excreted the residues all inside  one cell. They would swallow other smaller cells for that extra nutrition required to grow big. They did not have separate liver, kidney or brain ideas for special functioning. Because all these specialisation demanded more energy. These cells would grow till their size outstripped their energy source and die.

Evolution made provision for the extra energy through Mitochondria

One day, one big energy-starved cell swallowed another small cell. But this small cell inside the big cell did not die . Instead, it survived inside the predator and lived in a symbiotic relationship with its parent. The big cell provided nutrition to it. The small one cooked and provided extra energy to the mother cell. This symbiotic system allowed the cells so much extra energy that now the big cell could think of specialising into liver, fat cells etc. Huge multicellular organisms like us evolved after this singular phenomenon. This must be the crucial step that evolution took before creation of all the flora and fauna of nature. These swallowed organism became part  and parcel of the multicellular thing and are known as mitochondria. They provided extra energy so that the mother organism can specialise and form liver cells, brain cells or kidney cells.

Mitochondria is from mother- 100%

While we inherit our genes from both our parents, the mitochondria is inherited exclusively from the mother. Our mother has inherited her mitochondria  from her mother.

( We also know that the mitochondrial DNA of all the 8 billion human beings on our planet can be traced back to one common ancestor, a black lady who lived million years ago, by the big lakes, deep inside the forests of Africa.)

Mutations can affect the DNA of Mitochondria

Mitochondrial DNA like any other genes can mutate during multiplication leading to a defective gene. If that defect seriously hampers power supply to the new cells, that cell and the baby will die. That defect in the mother will also be passed on to all her offsprings, both sons and daughters. This is 100%, As father can’t share it to their babies.

 Mitochondrial diseases are incurable and can be fatal within days or even hours of birth.

Coenzyme Q, a neutraceurical is an essential item for the functioning of mitochondria. It is abundantly found in young children. As we age, the level of CoQ falls in various tissues. When the level of CoQ is low in brain, the mitochondria inside our brain cells fail to generate sufficient energy. As a result, their performance is slowed down. This reflects as dementia or Alzheimer disease.

When the level of CoQ 10 falls inside our heart cells, the energy production of heart cells is compromised and the pumping action of heart fails. Today, CoQ 10 is an important supplement in the treatment of heart failure.

Statins are a popular cholesterol lowering drugs. Unfortunately, in our body both cholesterol and Coenzyme Q share a common production path. Use of long term statins reduces the CoQ level in our body over a period. This leads to decreased generation of energy in our body. Hence people feel lack of energy and unusual tiredness after continuing Statin drugs for few months.

Can Coenzyme Q reverse the aging process ?

Unlike our nuclear DNA, the genes inside our mitochondria lack a robust repairing technology. With advancement of ages, the mitochondrial genes mutate and degenerate completely. Mitochondrial theory of Aging states that as we grow old, mitochondria in various tissues degenerate and stop giving energy output.

Today a cocktail of CoQ 10, ALA and Carnitine, all the three which help in the energy production inside mitochondria is being promoted as the perfect anti aging  mixture. Only time will tell their efficacy.

In memory of the proverbial king Yayati in the Mahabharat who was blessed with youth though temporarily  long after he has reached old age, CoQ 10 can be termed as the futureYayati molecule.

It seems sound theory that supplement of Coenzyme Q can enhance energy production by mitochondria. But can it be able to reverse the aging process.

Much research is going on this line. But there is no harm in taking an extra dose of Coenzyme Q till scientists come out with a definite answer.

Animal Liver, organ meat, fish and germinating seeds are rich source of CoQ 10.

Yesterday, the first UK baby was born who inherits genes from 3 parents.  The mother has mitochondrial defect.  While it received its X chromosome and Y chromosome from its biological parents, the baby received its healthy mitochondria from a third parent. 

Published by Dr. Ramakanta

Pediatrician and occasional blogger

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