what has happen to this kid
- 03-04-2009, 09:48 AM
what has happen to this kid
any new info on this kid any new info on ace-031 amg-745 myo-029
Rare condition gives toddler super strength
Updated Wed. May. 30 2007 9:44 AM ET
ROOSEVELT PARK, Mich. --
Liam Hoekstra was hanging upside down by his feet when he performed an inverted sit-up, his shirt falling away to expose rippled abdominal muscles.
It was a display of raw power one might expect to see from an Olympic gymnast.
Liam is 19 months old.
But this precocious, 22-pound boy with coffee-colored skin, curly hair and washboard abs is far from a typical toddler.
"He could do the iron cross when he was 5 months old," said his adoptive mother, Dana Hoekstra of Roosevelt Park. She was referring to a difficult gymnastics move in which a male athlete suspends himself by his arms between two hanging rings, forming the shape of a cross.
"I would hold him up by his hands and he would lift himself into an iron cross. That's when we were like, 'Whoa, this is weird,'" Hoekstra said.
Liam has a rare genetic condition called myostatin-related muscle hypertrophy, or muscle enlargement. The condition promotes above-normal growth of the skeletal muscles; it doesn't affect the heart and has no known negative side effects, according to experts.
Liam has the kind of physical attributes that bodybuilders and other athletes dream about: 40 percent more muscle mass than normal, jaw-dropping strength, breathtaking quickness, a speedy metabolism and almost no body fat.
In fitness buffs' terms, the kid is ripped.
"We call him The Hulk, Hercules, the Terminator," his mother said.
Liam can run like the wind, has the agility of a cat, lifts pieces of furniture that most children his age couldn't push across a slick floor and eats like there is no tomorrow -- without gaining weight.
"He's hungry for a full meal about every hour because of his rapid metabolism," Dana Hoekstra said. "He's already eating me out of house and home."
Liam's condition is more than a medical rarity: It could help scientists unlock the secrets of muscle growth and muscle deterioration. Research on adults who share Liam's condition could lead to new treatments for debilitating ailments such as muscular dystrophy and osteoporosis.
If researchers can control how the body produces and uses myostatin, the protein could become a powerful weapon in the pharmaceutical arsenal. It also could become a hot commodity among athletes looking to gain an edge, perhaps illegally, on the competition, experts said.
For Liam, the condition has one potential drawback: Infants and toddlers need some body fat to feed brain growth and the development of the central nervous system.
Without adequate body fat, a child's growth can be stunted and the central nervous system can be impaired, said Dr. Erlund Larson, an internist at Hackley Hospital who is familiar with Liam's condition.
That Liam appears to be thriving, physically and mentally, is almost as amazing as his feats of strength. The product of a troubled mother who gave him up for adoption at birth, Liam was born with a suite of medical problems.
The fact that Liam was adopted by a physician assistant's family hundreds of miles from his birthplace -- a stable family with the knowledge and means to give him all the food, nurturing, horseplay and love he needs to thrive -- might be the most miraculous part of his story.
"God works in mysterious ways," said Neil Hoekstra, Liam's adoptive father.
Myostatin-related muscle hypertrophy was first documented in beef cattle and mice in the late 1990s, according to scientific literature.
In 1997, researchers at Johns Hopkins University Hospital in Baltimore determined that Belgian Blue cattle, an unusually muscular breed, had mutations in the gene that produces myostatin. Those scientists also produced muscular mice by deactivating the rodent version of the myostatin gene, according to scientific journals.
The first human case was documented in 2000, in a German boy, but wasn't reported in medical literature until 2004. The condition is so rare in humans that scientists don't know how many people have it, said Dr. Kathryn R. Wagner, a genetics expert at Johns Hopkins.
A genetic mutation prevents some people from producing myostatin. Those individuals can have twice the normal amount of muscle mass, according to medical literature.
In Liam's case, the myostatin his body produces is rejected by muscle cells. He and others with his condition can have up to 50 per cent more muscle mass than the average person, experts said.
The result of both types of myostatin-related muscle hypertrophy generally are the same: above average growth of skeletal muscles, incredible strength, a warp-speed metabolism and minimal body fat.
"Liam's never had any body fat," his mother said. "The only fat he has is in his cheeks."
The so-called myostatin blockade has generated tremendous interest in the bodybuilding community. Some nutritional supplements claim to block myostatin, but researchers have said the claims are not scientifically valid.
"If the myostatin protein is knocked out, muscles grow and rejuvenate much more quickly," Dr. Larson said. "It has potential for great abuse in the future as the new steroid."
For Liam's parents, the most pressing challenge is feeding the boy enough protein every day to fuel his body's high-performance motor. The wiry but muscular toddler eats six full meals per day and still struggles to gain weight.
Dr. Larson, the first physician to suspect Liam had myostatin-related muscle hypertrophy, said he was amazed by the toddler's strength.
"He was able to grab both of my hands and nearly do an iron cross," Dr. Larson said. "This is not something that happens for most men, ever, and here is this kid with this kind of power."
Larson said Liam's strength gives him a huge edge over other children, physically and in terms of self-confidence.
"When you've got that kind of power and that kind of strength, the world is open to you," Larson said. "He's agile because he's so strong -- when you've got that incredible power as a kid you're going to try a lot more things."
Liam's father, a die-hard University of Michigan fan, already is dreaming big things for his adopted son.
"I want him to be a football player. He could be the next Michael Hart," Neil Hoekstra said, referring to U-M's star running back.
Liam was born four weeks early and had a small hole in his heart. He also had eczema, enlarged kidneys, was lactose intolerant and had severe stomach reflux that made him vomit several times each day, his mother said.
No one knew then that the baby was among the few people known to have myostatin-related muscle hypertrophy.
Dana Hoekstra said her suspicion that Liam was physically different quickly intensified. Two days after he was born, Liam could stand up and support his weight if someone held his hands to provide balance, she said.
His heart and kidneys healed within a few months, but it took 18 months before he stopped throwing up daily.
Liam's muscular thighs at 5 months of age gave him the appearance of a miniature Lance Armstrong. By 8 months, Liam was doing pull-ups and, a month later, climbing up and down stairs, his mother said.
What really amazed his parents was the way Liam fell.
"When he fell backward, he would land on his butt, but he never hit his head on the ground," Dana Hoekstra said. "His stomach would tense up and he would catch himself before his head hit the ground. You could see his stomach muscles. He had a little six-pack."
Liam has given his mother a black eye and once punched a hole in the plaster wall during a tantrum. "That's called attitude," his mother said.
After a series of stunning physical exploits, Dana Hoekstra's father -- retired Muskegon attorney Darryl Cochrane -- told Dr. Larson about the boy.
"Grandparents like to brag and Darryl was bragging about how powerful this kid was," Dr. Larson said. "I had to see for myself."
Dr. Larson said Liam exhibited phenomenal strength.
"When I saw him I knew he had some condition," said Dr. Larson, who considered it "a wild longshot" that Liam could have myostatin-related muscle hypertrophy.
After Dr. Larson observed Liam, the boy's pediatrician referred the toddler to the genetics clinic at Spectrum Health in Grand Rapids. Doctors there said Liam was well below average for height, weight and head circumference.
But they noted "significant hypertrophy (enlargement) by the Hoekstras. The diagnosis: Myostatin-related muscle hypertrophy.of his leg, calf and arm muscles as well as increased strength," according to medical records provided
The doctors at Spectrum said Liam likely inherited the condition from his biological father, who was reported to be unusually strong, according to medical records.
An ultrasound performed on Liam when he was 14 months old revealed he had 40 percent more muscle than average, Dana Hoekstra said.
Liam's condition also caught the attention of Johns Hopkins researchers who were studying myostatin-related muscle hypertrophy.
A blood test determined that Liam did not have the genetic mutation that blocks all production of myostatin. Rather, he has the myostatin blockade, his mother said. His is one of roughly 100 known cases in the world, according to experts and medical literature.
Researchers at Johns Hopkins wanted to include Liam in a study of people with the condition. When they found 100 adults to participate, Liam was no longer needed. That was a relief for his parents, who did not want to subject Liam to the painful muscle biopsy that would be required of everyone in the study.
Dana Hoekstra said she was prepared to allow Liam to be part of the Johns Hopkins study if it could have led to new treatments for muscular dystrophy patients.
For now, the Hoekstras are content to let Liam lead a normal life. They have no plans to take the advice of friends who have jokingly suggested they hire an agent for Liam to line up pro sports deals or modeling contracts.
"It's great that he's going to have some extra muscle mass, but I don't want him to be viewed as some kind of freak," his mother said.
Dr. Larson said Liam shouldn't be viewed or treated differently than other children.
"He's a normal kid. He's just got that lucky twist," Dr. Larson said. "It's going to be fun to watch him grow."
You can drive to the gym and wonder how you got there or you can consciously take note of the journey and enjoy the drive for the thing that it is.
- 03-04-2009, 10:18 AM
Lucky kid, aside from the hole in his heart, exzema, kidney problems and vomiting.
This is the condition that Flex Wheeler is said to have.
This also happens to dogs. My neighbor had a pit with Myosatin deificiency. It ripped off the fender off our neighbors Ford Expedition. He's ridiculousy muscular.
Just search Myostatin deficiency in google and look at the crazy pictures.
03-04-2009, 12:54 PM
03-05-2009, 12:06 PM
03-06-2009, 12:28 AM
What Is Myostatin?
Myostatin is a secreted protein that acts as a negative regulator (inhibitor) of skeletal muscle growth. Myostatin is a member of the transforming growth factor beta (TGF-B) superfamily of growth factors and is technically referred to as growth and differentiation factor 8 (GDF-8). Myostatin actually inhibits the growth of skeletal muscle by downregulating almost every myogenic (muscle building) factor known to induce skeletal muscle growth. Myostatin also influences special stem cells known as satellite cells away from myogenesis (the formation of new muscle cells) and toward adipogenesis (the formation of new fat cells). Myostatin is almost certainly the most powerful repartitioning substance that is produced in mammals and methods that will be used to suppress this protein will change the world forever.
Cattle with a myostatin deficiency accumulate an impressive amount of muscle tissue.
Myostatin And Humans
Have you ever seen a muscular individual that can eat whatever they want and not get fat? Are you envious that this person doesn't even have to exercise to look the way they do? Chances are this person was born with some form of myostatin deficiency. The myostatin protein was only discovered in 1997 and the identification of individuals with a deficiency of this protein has not occured until recently. While there are certainly many individuals that possess a myostatin deficiency there are only a few cases where a myostatin mutation has been detected in humans. The infant in the photo below is one of them.
An unidentified German male infant with a myostatin deficiency.
This child will grow up and possess enhanced skeletal muscle mass and super endurance. It is probable that many professional athletes today possess some form of a myostatin deficiency that allows them to excel in their chosen sport. Obviously not everyone was fortunate enough to be born with a myostatin mutation, however the future holds promise for a number methods to suppress myostatin and provide numerous benefits for those that undertake one these therapies.
Myostatin And Obesity
Americans spend billions and billions each year on ways to lose weight. Weight-loss drugs and supplements, special diets, and even surgical interventions (liposuction and gastric bypass) are utilized in an attempt to win the battle of the bulge. As I mentioned earliar the suppression of myostatin can induce a powerful repartitioning effect away from fat deposition and towards skeletal muscle accretion even in animals fed a high fat diet. Once an effective method for controlling myostatin is made available to the public the weight loss industry will go bankrupt. No more weight-loss pills, no more weird diets and no more expensive surgeries will accompany this revolution. This is not an exagerration, true myostatin suppression will make these measures obsolete. Some of you (especially women) may be concerned that too much muscle will be gained by using a myostatin inhibitor. This is not likely to be an issue as there will be a means to modulate and not eliminate myostatin activity.
Myostatin suppression and other future therapies will make obesity a thing of the past.
Myostatin And Muscle
By now it should be clear that myostatin suppression will induce skeletal muscle growth, but at this point I want to clear up a popular misconception. When I attempt to describe the benefits of myostatin suppression to anyone the inevitable comparison to anabolic steroids is made. You can think of an anabolic steroid like testosterone as the "gas" and the myostatin protein as the "brake" in relation to skeletal muscle growth. Anabolic steroids are able to enhance skeletal muscle growth because they overcome the inhibitory influence of myostatin on this process. The reason that myostatin suppression is an effective method for building new skeletal muscle is because you body produces it's own growth factors (testoterone, estrogen, Insulin, GH, IGF-I, HGF, FGF, IL-4, IL-15, PGF2a, others) and with no "brake" (growth arrest) in place these growth factors can proceed uninhibited. A major difference between anabolic steroid administration and myostatin suppression boils down to the side effects. Anabolic steroids induce numerous side effects while myostatin inhibition appears to be largely void of any serious side effects. Myostatin suppression therapy will be sought after by a large percentage of American men (almost all of them) that want to develop a lean muscular body with little or no exercise. Women will also want the benefit of added strength and muscle mass that accompanies this therapy and modulation of myostatin activity will be able to prevent gross muscle hypertrophy in females that seek this treatment.
Few people have the potential to build muscle like Arnold. Myostatin suppression therapy will help change that.
Myostatin And Disease
At this point in time all the research on myostatin suppression is focused on the treatment of muscle wasting diseases. Muscular dystrophy, AIDS, sarcopenia, cancer cachexia, and other states where extreme skeletal muscle breakdown is an issue could in part be treated through myostatin inhibition. Suppression of the myostatin protein will not only help improve our appearance, it will actually save lives. There are numerous diseases that will benefit from myostatin inhibition and in fact this will be one of the biggest markets for this type of therapy.
Myostatin suppression therapy will help patients recover from potentially fatal muscle wasting diseases.
Myostatin Suppression Therapies
Now that the benefits of myostatin suppression are understood the question is how best to inhibit the activity of this protein. There are a number of supplements that claim to be myostatin inhibitors, but in reality all of these products are a scam and have never demonstrated an in vivo ability to inhibit myostatin. It may be that true myostatin suppression is only possible with the development of drugs designed for this purpose. At this time there are three pharmaceutical corporations that are developing these specific myostatin inhibitors, Acceleron Pharma Inc, Amgen, and Wyeth Pharmaceuticals.
Myostatin control will allow men and women to shed fat and build muscle.
Acceleron Pharma has produced a powerful myostatin inhibitor based on ACVR2B. The designation given to this drug is ACE-031, the first in a series of myostatin inhibiting substances that this company is working on. Mutant activin type II receptors inhibit myostatin activity and ACE-031 utilizes this pathway to suppress myostatin.
Amgen is developing AMG 745, a peptide based drug designed to bind with and suppress the activity of myostatin. AMG 745 may be similar to the myostatin propeptide. The myostatin propeptide is produced in the body and binds with myostatin thereby reducing the availability of the protein to available receptors.
Wyeth pharmaceuticals has developed MYO-029, an antibody that binds to and inhibits myostatin activity. The Wyeth myostatin antibodies should be approved in the near future for use in patients with muscular dystrophy.
An actual vaccine has been produced that would allow for the endogenous production of antibodies that inhibit myostatin. A myostatin vaccine may never be used in humans but it would be wildly popular with those individuals that raise livestock for a living. Click the link below for an abstract about the myostatin vaccine.
Follistatin and follistatin related gene (FLRG) are two other proteins that are produced by the body that also bind with myostatin and act as inhibitors of the protein. While there is no question that follistatin and FLRG act as endogenous myostatin inhibitors, the overexpression of these two proteins has been associated with reproductive organ abnormalities in both males and females. This may be the reason that these companies are not pursuing the development of myostatin suppressing drugs that based on these two proteins.
The future of myostatin suppression therapies
The myostatin inhibiting therapies that are being developed, as mentioned before, are for those that are suffering from a disorder that affects the development of skeletal muscle or for those experiencing an illness that induces skeletal muscle wasting. It is not likely that the FDA will approve these drugs for cosmetic enhancement (fat-loss and muscle accretion) anytime soon. However, effective myostatin inihibiting therapies will be sought after as soon as the public becomes aware of how these therapies can improve their body composition. If the American public is denied these therapies for cosmetic use by th FDA then a lucrative black market will emerge to fill the demand. Other nations in the world will not have to grapple with this issue, as these therapies will receive quick approval for the cosmetic use of these drugs. It is inevitable that the widespread availability of these drugs in other countries will make it easier for Americans to obtain these myostatin inhibitors via mail order. Independant individuals and companies will also cater to this black market demand. The genie is out of the bottle and he's not going back in. Every now and then a technology comes along that has a ripple effect on multiple industries and the various myostatin inhibiting therapies is one such technology. The revolution is about to begin.
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03-06-2009, 02:30 PM
03-06-2009, 06:45 PM
03-06-2009, 06:49 PM
Yeah, this condition (and related ones) pop(s) up around here every few months. Dogs look really crazy:
EDIT: I just saw that there's another thread about this same kid.People mistake her for a pitbull with a pinhead, but Wendy the whippet is one rare breed.
So rare that the Central Saanich dog recently graced the New York Times. She also had several of her photos shown on The Today Show, all because of a rare genetic mutation that has led to her being the Incredible Hulk of dogs.
Wendy is a 27-kilogram rippling mass of muscle. Forget the so-called six-pack stomach: Wendy has a 24-pack. And the muscles around her neck are so thick, they look like a lion's ruff.
"People have referred to her as Arnold Schwarzenegger," says doting owner Ingrid Hansen, stroking Wendy's sleek black coat and white chest.
Wendy was recently part of a genetics study done in the U.S. on mutation in the myostatin gene in whippets, which resemble greyhounds in appearance. The National Institute of Health study reported that whippets with one single defective copy of the gene have increased muscle mass that can enhance racing performance in the breed, known for speeds up to 60 kilometres an hour.
But whippets with two mutated copies of the gene become "double-muscled," like Wendy. It has been seen before in one human, and also in mice, cattle and sheep, says the study.
The uber-muscled whippets are called "bullies," not because of their nature -- Wendy likes nothing better than a good back scratch and isn't shy about sitting in your lap to ask for one -- but because of their size. She's about twice the weight of an average whippet, but with the same height and small narrow head -- and the same size heart and lungs, which means she probably won't live as long as normal whippets.
Hansen has had Wendy, now four, since she bought the dog from a Shawnigan Lake breeder when she was eight months old.
Wendy landed in clover. She lives on an acreage, runs around with other dogs and horses, sleeps on Hansen's bed and pretty much anywhere else she wants to.
People are often afraid when the muscle-bound dog runs up to them on her dainty whippet-thin legs, but they soon realize she's friendly, Hansen said.
© (c) CanWest MediaWorks Publications Inc.
Interesting topic for sure:
Baby with super human strength
RcB Since 09-06-2011 20:55 EST, Post 49
03-06-2009, 07:25 PM
03-06-2009, 07:38 PM
03-06-2009, 10:08 PM
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