The Ecuadorian dwarf community 'immune to cancer and diabetes' who could hold cure to diseases

Laron dwarfs seem to be immune to cancer and other diseases



Sufferers of Laron lack a hormone called IGF-1, linked to cancer

Scientists developing drug they hope will mimic genetic defect in Laron



They are completely free of two of the most debilitating illnesses - diabetes and cancer.



Now scientists hope a community of dwarfs living in a remote corner of Ecuador could hold the key to a cure for both.

The Laron dwarfs, who have a condition believed to be caused by inbreeding, appear to be completely immune to cancer and other age and lifestyle associated diseases.

People who inherit the genetic defect are in perfect proportion but grow only to an average height of 4ft.



For more than 24 years, endocrinologists Jaime Guevara-Aguirre (top left) and Arlan Rosenbloom (top right) have tracked a population of Ecuadorians dwarves who seem to be immune to cancer Jaime Guevara-Aguirre (pictured in 2009 with some participants in his research) was the first to identify a population of Ecuadorians with Laron syndrome

Now scientists are developing a drug which they hope will do artificially what the genetic defect in Laron syndrome does naturally - protect against DNA damage that fuels cancer growth.



There are around 300 people in the world with the condition Laron dwarfism, a third of whom live in remote villages in Ecuador’s southern Loja province.

Sufferers of Laron lack a hormone called Insulin-like Growth Factor 1, or IGF-1.



It is IGF-1, not growth hormone, that stimulates the cell to grow and divide to form new cells.

In ordinary humans, too much of the hormone can lead a person to develop breast, prostate or bowel cancers at an early age, Discovery Magazine reports.



Studies of the Laron group showed that while they had high levels of growth hormone, their cells were not generating IGF-1.



An Ecuadorian man with Laron syndrome (pictured with his children in the early 1990s). He lived free of cancer and diabetes to 87, a decade longer than the average Ecuadorian. He was killed in a car accident in 2012

Dr Jaime Guevara-Aguirre, a hormone expert from the Ecuadorian Institute of Endocrinology, and Dr Valter Longo at the University of Southern California have been studying the Laron group for the best part of two decades.



They believe that having less IGF1 could mean suffering less of the DNA damage that occurs in all of us when we age and which leads to cancer.



They are also studying whether the Laron dwarfs could hold the key for the prevention of diabetes, as d espite a tendency to obesity due to overeating and a poor diet, it is another condition to which they appear immune.

Guevara-Aguirre was inspired to study Laron syndrome after growing up in the Loja province in southern Ecuador, where he often saw short people known locally as pigmeitos - loosely translates as pygmies - living in small towns

He told Discovery Magazine: 'I had seen maybe 20 of them on the streets when I was young.'

Enlisting the help of a family acquaintance Guevara-Aguirre, visited the provinces of southern Ecuador, not too far from where he had spent parts of his childhood, to find more.

He met with families, asked questions, and took blood samples, discovering more and more cases each week .



By late 1989, they had enough cases— 20 total, 19 of them women—to publish an article on the 'Little Women of Loja' in NEJM, which recognised the growth hormone deficiency in the inbred people.



But it is Dr Longo who has taken the first step into developing anticancer and antiaging drugs with lessons learned from Laron syndrome .



In 2008 he founded DSR Pharmaceuticals with the aim of developing a pill that blocks the growth hormone receptor.



It is hoped the drug will do artificially what the genetic defect in Laron syndrome does naturally - protect against DNA damage that fuels cancer growth.



A more expensive injection form of the drug was discovered a decade ago.



It has since been approved by the regulatory body the Food and Drug Association to treat individuals with acromegaly, a condition in which there is too much growth hormone in adults, resulting in abnormal growth of body tissues, especially hands, feet, and face.



Now Longo believes that it might also be useful in the treatment of cancer.



THE ACCIDENTAL DISCOVERY WHICH 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 growth and sexual development disorders. His earliest patients included three young siblings—two boys and a girl— all with severely stunted growth. They were all obese, with thin hair, pronounced foreheads, and a collapsed nasal bridge, and their grandparents were first cousins.

Laron assumed they had a severe shortage of growth hormone, but some years later, after new blood tests were developed, it transpired the levels of growth hormone were abnormal—they were extremely high.

By the mid-1960s he had identified 20 more such 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 tiny locks for hormones, Discover Magazine reports.

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

But while studying the cells of his subjects, Laron found that normal growth hormone failed to bind to its associated receptor, suggesting that they were damaged.

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







