Home Australia Rare type of dwarfism could be key to longer life, scientists say

Rare type of dwarfism could be key to longer life, scientists say

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Doctors Jaime Guevara-Aguirre (back left) and Valter Longo (back right), photographed with several of the Laron study participants at the USC Leonard Davis School of Gerontology in Los Angeles. The two researchers have followed people with Laron syndrome for years to find out what kind of advantages their rare genetic syndrome gives them.

A rare form of dwarfism has been found to protect people from cancer and diabetes, suggesting the gene could be the key to a longer life.

Known as Laron syndrome, people with this condition are about four feet tall because their bodies cannot make use of their own growth hormone produced in the brain, but they have a similar average life expectancy to those without the condition. disorder.

Scientists who have followed a group of adults with Laron syndrome for almost 20 years have discovered how their rare disease can be a gift, protecting them from aging-related diseases and potentially paving the way for anti-aging treatments.

In the latest study on this group, a team of scientists from California and Ecuador found that people with Laron syndrome have lower blood sugar, insulin resistance, and blood pressure compared to their relatives who do not have the condition. .

Doctors Jaime Guevara-Aguirre (back left) and Valter Longo (back right), photographed with several of the Laron study participants at the USC Leonard Davis School of Gerontology in Los Angeles. The two researchers have followed people with Laron syndrome for years to find out what kind of advantages their rare genetic syndrome gives them.

Ecuadorian Maritza Valarezo (right) and her sister Lugartda (left), both have Laron syndrome. There are only between 400 and 500 people worldwide who suffer from this rare genetic disease.

Ecuadorian Maritza Valarezo (right) and her sister Lugartda (left), both have Laron syndrome. There are only between 400 and 500 people worldwide who suffer from this rare genetic disease.

Study participants were also found to have smaller hearts than their relatives, thinner carotid artery thickness and lower rates of arterial plaque, the substance that can narrow arteries and cause heart attacks, heart failure or blood clots.

Doctors first identified Laron syndrome among adults with dwarfism living in a remote corner of Ecuador. It is also known as growth hormone receptor deficiency (GHRD).

People with Laron syndrome actually tend to have high levels of growth hormone, but their genetic mutation causes their body to be unable to produce the hormone’s target.

This target, called a receiver, is like a lock.

When the study’s co-senior author, Dr. Jaime Guevara-Aguirre, began studying Laron syndrome, he was surprised that the condition seemed to protect against cancer.

After all, higher levels of growth hormone are generally linked to cancer, and this relationship has been reliable in most people.

Guevara-Aguirre, an endocrinologist at the Universidad San Francisco in Quito, Ecuador, has been studying Laron dwarfism for more than 20 years and previously told DailyMail.com: ‘We have found that people with Laron simply do not develop cancer.

“Cancer can be detected in their relatives of normal size, but never in my patients, not a single case,” he said.

Most people with Laron syndrome come from Ecuador, where the average height of men and women (five feet, six inches and five feet 1/2 inch) is among the shortest in the world.

For this reason, these people do not tend to consider themselves particularly short.

Women with Laron are on average around four feet tall and men are typically around four feet six inches tall.

But what many of them do realize is that they have a special biological gift.

‘Since I was a teenager I saw my height as (an opportunity) to develop, the growth problem is part of my personality’, Nathaly Paola Castro Torres said Cnn in Spanish.

“I’m also very lucky because my body actually protects me a lot from diseases that other people suffer from every day,” he added. “This height, while being a limitation, is also a blessing.”

Insulin-like growth factor 1 (IGF-1) is produced in the liver. In people with typical genetics (left), growth hormone from the brain's pituitary gland signals the liver to produce more IGF-1, which can contribute to aging and age-related diseases. But in people with Laron syndrome (right), despite high levels of growth hormone, their liver produces much lower amounts of IGF-1, which protects them against heart disease.

Insulin-like growth factor 1 (IGF-1) is produced in the liver. In people with typical genetics (left), growth hormone from the brain’s pituitary gland signals the liver to produce more IGF-1, which can contribute to aging and age-related diseases. But in people with Laron syndrome (right), despite high levels of growth hormone, their liver produces much lower amounts of IGF-1, which protects them against heart disease.

Maritza (left) and Lugartda Valarezo (right), in their kitchen in Quito, Ecuador, in 2014. The Valarezo sisters are patients of the study's lead co-author Jaime Guevara-Aguirre, who has studied Laron syndrome for more than two decades.

Maritza (left) and Lugartda Valarezo (right), in their kitchen in Quito, Ecuador, in 2014. The Valarezo sisters are patients of the study’s lead co-author Jaime Guevara-Aguirre, who has studied Laron syndrome for more than two decades.

Because of their resistance to chronic diseases, people with Laron syndrome have been the subject of great interest by doctors and scientists seeking treatments for diseases related to aging.

“We have shown in studies that people with Laron have a very low incidence of cancer, diabetes and cognitive decline,” said study co-senior author Dr. Valter Longo, a professor of gerontology at the University of Southern California.

‘Brain scans have shown that they have younger brains, the equivalent of someone 20 years younger. I have yet to see a case of Alzheimer’s in this population.’

Some even claim that studying these patients will lead to cures for cancer and diabetes.

“This doesn’t mean they are immune to these diseases, but people with Laron syndrome certainly seem to be very protected,” Longo said of the latest study on heart health. “That’s how powerful this mutation appears to be.”

According to the new study, the key appears to be a hormone called insulin-like growth factor 1 (IGF-1).

This chemical is mainly produced in the liver.

In people with typical genetics, growth hormone from the brain’s pituitary gland instructs the liver to produce more IGF-1, which promotes normal wound healing and other metabolic processes.

However, elevated levels of IGF-1 may contribute to aging and age-related diseases.

In people with Laron syndrome, despite high levels of growth hormone, their liver produces much lower amounts of IGF-1, which appears to protect them against heart disease.

In mice, IGF-1 deficiency has caused heart disease, so the results of the new study were somewhat unexpected.

Researchers also said that low levels of IGF-1 in the blood of people with Laron syndrome contribute to reduced risk of cancer and diabetes, but more research is needed to determine the details of that connection.

“Although the population analyzed is small, together with studies in mice and other organisms, these human data provide valuable information about the health effects of growth hormone receptor deficiency and suggest that medications or dietary interventions that cause similar effects could reduce the incidence of disease and possibly prolong longevity. long saying.

He study was published in the magazine Medicine.

The accidental discovery that scientists say could lead to a cure for cancer

In the late 1950s, Israeli endocrinologist Zvi Laron began studying patients, some with childhood diabetes and others with disorders of growth and sexual development.

His first patients included three younger siblings, two boys and a girl, all severely stunted. They were all obese, with thin hair, a pronounced forehead and a collapsed nasal bridge, and their grandparents were first cousins.

Laron assumed they had a severe shortage of growth hormone, but a few years later, after new blood tests were developed, it turned out that the growth hormone levels were abnormal: they were extremely high.

By the mid-1960s he had identified 20 more people. He reasoned that either they had defective hormones or something was wrong with the cells that respond to those hormones.

Every cell in the body contains receptors, which act like little locks for hormones, Discover magazine reports.

When a hormone finds a matching lock, it binds to the receptor and causes the cell to do something, like grow or divide.

But while studying his subjects’ cells, Laron discovered that normal growth hormone failed to bind to its partner receptor, suggesting that they were damaged.

In 1984, he published a paper showing that the short stature of people with Laron syndrome is due to their inability to respond to growth hormone, no matter how much of it they have floating around.

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