Scientists Discovered the 'Werewolf' Gene....

Scientists from China and the USA have identified a genetic region linked to 'werewolf syndrome'.

Researchers from the University of Southern California, USA, working with scientists Prof. Dr. Xue Zhang of medical genetics at the Peking Union Medical College from Beijing, China, studied the genome of a Chinese man with ‘Hypertrichosis’ and identified a defective region of the X chromosome they believe is linked to the condition. As per the  discovery the man had an extra stretch of DNA known as an 'inserted-sequence' in a region very close to SOX3, a gene known to be involved in hair growth. The inserted sequence had altered the activity of SOX3, leading to excessive hair growth.

The finding was also confirmed in a Mexican family, in which several members had ‘Hypertrichosis’. But in this case, although the location of the insertion was the same, the 'inserted sequence' itself was not the same as in the Chinese man. This suggests it is the region of the X chromosome that is more important in controlling hair growth, rather than the exact defect itself.

This study may lead to improvements in the treatment of Hypertrichosis, but the researchers also hope the discovery could lead to treatments for male pattern baldness. Prof. Pragna Patel, who is also the part of the stufy, from the University of Southern California told the Daily Mail: 'If in fact the inserted sequences turn on a gene that can trigger hair growth, it may hold promise for treating baldness'.

The study was published in the American Journal of Human Genetics.

Source: www.huffingtonpost.com/2011/06/07/wolf-boy-larry-gomez-wolf-boy-werewolf-syndrome_n_872229.html 

Hypertrichosis & Genetics

www.videos.howstuffworks.com/discovery/30742-one-step-beyond-hypertrichosis-video.htm

Meet Wolf Sisters !!!...........

Meet Supatra Sasuphan..... World's Hairiest Girl

Larry & Danny......

'Wolfman' Larry Gomez

Severe Hypertrichosis is quite rare, almost certainly due to unknown genetic defects, and can result in excessive or animal-like hair on both face and body.

In 1982, an American geneticist called Frank Greenberg of the Baylor College of Medicine discovered a Mexican family with a rare genetic mutation that causes fur like hair to grow all over their bodies. The family was segregated from Mexican society; they were forced to hide in their home in Loreto's town, and can only obtain work in the circus. All the 32 members in the Aceves family have this disease. They live in a mountain town named Zacatecas.

Victor "Danny" Ramos and Gabriel "Larry" Ramos are the most famous of the family. These brothers have excessive hair on their face and chest, almost looking like fur — more than 95% of their body is covered with hair. The only visible place they do not have this hair is around their eyes, mouth, and upper ears and hands.

In Larry & Danny’s family, there are at least five generations of people with ‘Hypertrichosis’ — about 20 affected persons are there in the family including men and women who are suffering with this rare genetic disorder of excessive facial and torso hair. The women are covered with a light-to-medium coat of hair while the men of the family have thick hair on approximately 98% of their body apart from their hands and feet.

Geneticist Dr. Luis Figuera, an expert in hypertrichosis at Mexico's Center for Biomedical Research, has studied people with the condition for more than 20 years. As per Dr. Figuera, Danny's grandmother does not have hypertrichosis, but she carries the mutation and passed it on to her children.

Danny's brother, Larry, born as Gabriel Ramos Gomz in Maxico but took his new name from a 1941 horror film ‘The Wolf Man’, had a son — Michael, now 11, who doesn’t have hypertrichosis whereas Danny passed this mutated gene on to his 7 yrs old daughter — Daniela.

Danny's cousins Lili and Carla, his sister Jamie and his daughter Daniela all have varying degrees of hypertrichosis.

Acquired Hypertrichosis - Types

Acquired hypertrichosis, unlike congenital syndrome, appears after birth. This particular syndrome might appear due to several reasons — side effects of certain drugs, person’s association with cancer and possible links with any form of eating disorder.  This type of syndrome is characterized by hypertrichosis lanuginose, generalized hypertrichosis, patterned hypertrichosis and localized hypertrichosis.

Acquired Hypertrichosis Lanuginosa : People with acquired hypertrichosis experiences rapid growth of lanugo hair on different parts of the body most especially on the face.

Acquired Generalized Hypertrichosis : Abnormal and unusual growth of unwanted hair occur on different body parts like the upper lips, legs, forearms, chin and cheeks.

Patterned Hypertrichosis : Rapid growth of unwanted hair follows a specific pattern.

Localized Hypertrichosis : As the name suggests, it is the localized or confined increase in the density and length of hair.

Congenital Hypertrichosis - Types

Before taking a look into the other live cases of ‘Hypertrichosis’ let’s take a dig to the different types of ‘Hypertrichosis’ —

Different Types of Werewolf Syndrome

Hypertrichosis is classified into 2 types — ‘Congenital Hypertrichosis’ and ‘Acquired Hypertrichosis’.

Congenital Hypertrichosis is characterized by the overproduction of fine, soft and non-pigmented hair and continues through life. This condition normally occurs due to some flaws at the course of pregnancy. The lanugo, which refers to the fine hair layer covering the fetus, normally falls off on the eighth month. If this fails to happen, the hair continues to grow. Congenital Hypertrichosis can be sub-divided into 5 types as follows

Naevoid Hyperthrichosis: in this case hair grows excessively on one part of the body surrounded by normal hair.

Hypertrichosis Lanuginosa: It is visible at the birth time with the infant covered in thin ‘lanugo hair’. In a normal condition, lanugo hair sheds before birth — but for a person suffering from congenital hypertrichosis lanuginosa, the lanugo hair remains even after birth.

Generalized Hypertrichosis: Overproduction of hair is visible on the upper part of the body and face. The palms, soles and mucous membranes, however stay unaffected.

Circumscribed Hypertrichosis: Thick vellus hair present on the upper extremities is associated with congenital circumscribed hypertrichosis. This type of syndrome is restricted to certain specific parts of the body, in this case the upper part of the body. Hair elbow syndrome is one of the examples of congenital circumscribed hypertrichosis. This syndrome develops with birth and grows with ageing, only to lapse at puberty.

Terminal Hypertrichosis: Terminal Hypertrichosis is defined by the presence of completely pigmented terminal hair, covering the entire body. The condition is accompanied by gingival hyperplasia. This term is very much relative to ‘Werewolf Syndrome’, due to the dark-thick hair that grows.

Julia Pastrana : After Death ... (5)

Theodore Lent was distraught. Julia had been the bank. Now the bank was closed! How he to live now as his source of income was had died?

He had a Eureka moment!

Why should the bank close?

He sent Julia’s corpse and his newborn son to Professor Sukolov of Moscow University for embalming. The process took 6 months but the results were amazing. Julia’s mummified remains looked lifelike. Prof. Sukolov put Julia and his son in the Anatomical Museum of the university where the attracted great crowds of people.

Was this the end of Julia Pastrana? No !!! Not at all !!!

Lent realised what had happened and much to the public horror, he took the awful and unbelievable steps to go to court to reclaim the bodies. He presented his marriage certificate to the American consul and Sukolov was forced to release the remains.

Lent tried to put the mummies on display in Russia but the authorities refused as they were outside the confines of a scientific institute. In 1862, Lent return to England to show Julia Pastrana again. The price was only a shilling and with the added attraction is the mummified infant — the exhibit was packed with onlookers. Inside it was said that the ‘Embalmed Nondescript’ stood dressed in one of her many dancing costumes while her son stood to her left ~ atop of a small pedestal, dressed in a sailor suit.

Julia Pastrana : “The Ape Woman” ... (4)

In late 1859, while in Moscow, it was discovered that Julia realized that she was pregnant.  This was a disaster. Not only would she have to take time off, and lose money, from the tours but Mr. Lent was panicked due to the thought of Julia’s death. And then where would that leave him? He Took her to many different Doctors who were afraid that she would not be able to have the child naturally because they believed her hips would be malformed, however Julia was more concerned that the baby should take after its father.

On March 20th 1860, Julia’s fears were confirmed when she gave birth to a hair covered baby boy. All Julia could think of was, please be healthy and not have a personality like your father. Sadly the child died after 35 hours.

Julia's time was coming to an end. Complications during the birth left her weak and drained. Only 5 days later, Julia was dead at the age of 26 only.

Julia Pastrana : “The Ape Woman” ... (3)

In 1857 when Julia toured London in one of the monster shows popular at that time, she attracted journalists, doctors, and scientific minds. Julia was very popular. It cost 3 shillings to see her in the Regent Gallery, compared to the 6 shillings that a Victorian labourer might earn in a week. Promoted by her new manager Mr. Theodore Lent, Julia was now billed as “The Nondescript” ~ suggesting that she was a unique species, perhaps “the missing link” between humans and the rest of the animal kingdom. Debate raged in the newspapers as to her origins and her appearance was described at length. In these articles, Julia is described as being very civilized and domestic.In addition to her native language, she also spoke Spanish and English quite well. She loved to travel, cook and sew.She willing gave herself to medical examination and was said to have an eager thirst for knowledge. These articles also seemed to emphasize that she was both happy and content with her situation and she did not covet wealth, though her ‘handler’ Mr. Lent surely did. During her performances in London, Julia sang romances in both Spanish and English and danced what are described as ‘fancy dances’ – likely traditional Spanish numbers.

After London Mr. Lent secured a tour of Berlin and in Leipzig, Julia played the leading role in a play called “Der curierte Meyer”. Following the play, the weekly magazine ‘Gartenlaube’ published an extensive interview with Julia — an article published with a fantastic life sketch by the artist H. Konig (pictured below).

The article consisted of Julia speaking on her tours of America and London and of the numerous marriage proposals she had received. She claimed to have turned down over 20 admirers because ‘they were not rich enough’. That was a response that the reporter suspected Mr. Lent had coached in the hopes of attracting a rich suitor.

That notion was short lived and Mr. Lent, worrying of losing his investment in Julia to rivals, married her in 1857. Julia was infatuated with her husband but as per evidence Mr. Lent was not a kind man. By the time they got to Vienna, she wasn't allowed out in the daylight, because Lent was embarrassed to be seen with her. As their tour continues through Poland and to Moscow, Mr lent became more and more aggressive.

Julia Pastrana : “The Ape Woman” ... (2)

In New York, Julia attracted the attention of many scientific minds and media moguls. One newspaper described her as ‘terrifically hideous’ and possessing a ‘harmonious voice’ ~ which gives evidence that she sang during her exhibition. One of the members of Medical society Dr. Alexander Mott who examine her and declared her ‘the most extraordinary beings of the present day’ and ‘a hybrid between human and orang-utan’.

After moving to Cleveland with another new promoter Mr. J. W. Beach, Julia began to turn the tide around and charming the public with her grace, she began to get very well known, even being invited to grand balls and military gala's. On one of these occasions, she was given the honor of dancing with the soldiers, who lined up to have the chance to dance with this unique but strange human being. The Misnomered Bear Woman — Julia again impressed with her grace and singing voice so much that she was put on exhibition by both the ‘Horticultural Society’ and the ‘Boston History Society’. While Julia was in Cleveland Dr. S. Brainerd declared her a ‘distinct species’. That analysis was quickly added to all subsequent promotional materials.

Julia Pastrana : “The Ape Woman” ... (1)

The prodigious (unnatural/abnormal) Julia Pastrana was known by many monikers during her life and perhaps just as many names in death. Both her life and her death were sad tales but they hold a very special place in sideshow history because, for a time, she was not considered a member of the human race.

Julia’s origins are shrouded in mystery. It is believed that she was born in 1834 to a tribe of ‘Root Digger’ Indians in the western slopes of Mexico. Julia was suffered from a rare inherited disorder — Hypertrichosis — not understood during the Victorian Age which caused her entire body to be covered in silky, black hair.  In addition to excessive hairiness over her body ~ predominately in the face ~ Julia also possessed a jutting jaw and swollen gums — huge teeth made her look positively like a monkey. In odd juxtaposition to her ape like features, Julia possessed great poise in her 4 ½ ft figure with a well developed a buxom.

Her documented career began in 1854 at the age of 20 years as she was exhibited in New York at the Gothic Hall on Broadway as ‘The Marvelous Hybrid or Bear Woman’. Her ‘handler’ was Mr. M. Rates who allegedly discovered the young Julia as a servant girl to the governor of Sinaloa, Mexico.

The 1st WOLFMAN !! — Petrus Gonzales (4)

Men seem to adjust to this genetic disorder more easily than women, as evidenced by the history of the Gonzalez family. Roman art historian Roberto Zapperi  (Zapperi  reconstructed the varied life of the hirsute man and his family by examining archives, art collections and libraries, and published a book of his findings) believes that "their excessive hair may actually have heightened the appeal of the males in the family, because it created the impression of exceptional virility."

After the death of French King Henry II, the members of the Gonzales family were given to other royal families, where they were gaped at and painted. 4 large paintings of the Gonzales family still hang in ‘Ambras Castle’ near Innsbruck, Austria. The faces of the subjects, offset by white ruffled collars, look like an evil trick of nature. Their bearing gives them the appearance of elegant human beings but the hair covering their faces suggests proximity to the animal kingdom.

After years of being gawked at, Enrico, the eldest son of Petrus Gonzales, managed to trick his master into allowing the family to live quietly in the small village of ‘Capodimonte’ on Lake Bolsena in Italy. He convinced his master ~ Cardinal Odoardo Farnese ~ that he and his family as wild creatures were magically drawn to a life in nature. Farnese, a religious man from Rome, was ultimately unable to resist Enrico's convincing but tall tale of the powers of animal instinct.

In the remote village, inhabited by only a few farmers and fishermen, Enrico brought together the widely dispersed members of his extended family, married healthy women several times and managed to achieve modest wealth as a businessman. His father, Petrus Gonzales, spent the last few years of his life in Enrico's idyllic village and died peacefully at the advanced age of about 80 — the exact date of Petrus Gonzales’s death is unknown but memorials to Gonzales appeared around 1635.

But the fate of the hirsute Gonzalez daughters is more reminiscent of the suffering of "ape woman" Pastrana. Like their father they also lived long lives but theirs was a hidden existence.

Petrus Gonzales (1618)

Petrus Gonzales & his family. From Top Left, clockwise: Petrus Gonzales, his wife, his son, his daughter.

The 1st WOLFMAN !! — Petrus Gonzales (3)

In 1573, at the age of 17, Petrus married a young French lady and by 1581 with whom he raised several children, including — three daughters (Maddalena, Francesca and the youngest Antonietta) and two sons (Enrico and …). All of his children shared his unique appearance and the entire family became the most sought after curiosity of the era.

In 1581 the family began a tour of Europe.

In 1582 their portraits were painted in Munich by the order of Duke Albrecht IV of Bavaria.

In 1583 the Gonzales family went to Basel where they were studied by the famed anatomist Felix Plater and he published a detailed account of the visit in his Observationum and further less detailed accounts followed the travels of the family until the early 1590’s.

The 1st WOLFMAN !! — Petrus Gonzales (2)

Petrus Gonzales was the 1^st described case of ‘Hypertrichosis’ aka Wolfman Syndrome in 1557.

In 1557, the first formal report published, written by Julius Caesar Scaliger, where Scaliger referred Petrus as ‘Barbet’ — a breed of shaggy dog.

A second report in the same year (1557) confirms the arrival of Petrus in Paris. The report also states that King Henri II ordered that the furry boy was to receive a formal education ~ not to be kind but rather out of curiosity ~ the King believed that Petrus was a savage and incapable of learning.

His progress was monitored closely and he proved the King quite incorrect by not only learning the basics of education but also becoming fluent in the noble gestures, etiquette and tact. He became quite fluent in the language of the affluent, Latin, and took to wearing splendid robes that actually further accentuated his furry covered face. It was in this way that Petrus became a sought after court guest, a prodigy royal dignitaries and ambassadors flocked to see. He became a great asset to the court of King Henri and was rewarded for his service.

The 1st WOLFMAN !! — Petrus Gonzales (1)

During a period of history which was very quick to peg the unknown as dangerous, wolf-boy “Petrus Gonzales” was lucky enough to encounter fascination rather than fear.

Nothing is known about the parents of ‘Petrus Gonzales’ as he was taken, as an infant, from his home in the Canary Islands to be presented to King Henri II in Pairs.

But what make king Henry II to be curious on Petrus ???

Petrus Gonzales’s entire body ~ including his face was covered in long, wavy hair and he was an immediate-medical sensation.

The Name……

The name ‘Ambras’ comes from a “Petrus Gonzales” — who in 1956, at the age of 12 was brought as a slave from Tenerife , Canary Islands to the court in France. He had a strikingly hairy face, married young and had 4 children ~ 3 of whom were born hairy. His daughter ‘Tognina’ and son ‘Arrigo’ passed on the trait. Royalty, who called Petrus the “man of the woods”, thought he represented a race of hairy people from the Canary Islands. He and his hairy sat for family portraits and one displayed at a castle near Innsbruck called ‘Ambras’, led to their notoriety as the ‘family of Ambras’ and eventually their condition as 'Ambras syndrome'. 

Portrait of Tognina Gonzales

Portrait of Antonietta Gonzales, by Lavinia Fontana, cover image of “The Marvelous Hairy Girls.”

Lifespan.....

The average lifespan is regular like people without hypertrichosis.

ONLY 50 Cases Known………

Statistics say ‘Hypertrichosis’ or the ‘Werewolf syndrome’ is so rare that it occurs in 1 out of 10 billion people.

There are 19 people is the world alive today with ‘Hypertrichosis’ out of 6.5 billion people, so it’s more like 1 in 340 million.

Only 50 cases have been reported since the middle age.

Werewolves !!!!!

WEREWOLVES !!!!!

When the moon is full a man sprouts hair over his body and is metamorphosed into a beast.

Did you believe in werewolves as a child? There is a bit truth to the myth. After college, one of the favourite thing me and my roomees loved to do was to watch a B grade horror movies about monster like Mummy, Dracula and Wolf-man. We would settle down after dinner and have a blast watching movies like ‘Wolf (1994)’, ‘The Wolfman (2010)’, ‘Bad Moon (1996)’, ‘Underworld (2003)’……… the list is pretty long. We thought it’s just a fantasy…fun & entertainment…..but wait……

That’s not a fantasy; it exits in reality……

I would have been much more horrified when I was watching these films if I realized there really was a rare, genetically inherited condition called the “werewolf syndrome” and how this “likely contributed to the ancient ‘Wolfman’ folklore according to the How Stuff Works article, “10 Bizarre Medical Conditions.”  

I am surprised by how – from ignorance- these poor people have been often labelled monsters. The IMDB database describes the 1941 movie, The Wolfman, starring Lon Chaney like this: “A practical man returns to his homeland, is attacked by a creature of folklore, and infected with a horrific disease his disciplined mind tells him cannot (sic) possibly exist.” But a horrific disease such as this does exist……just not exactly as it is described……

The Wolfman camouflaging disease known as “Hypertrichosis” or “Amber Syndrome”, is a very rare genetic condition. According to the Live Science article “Werewolf gene may explain Hair disorder” there are ‘fewer than 100 cases documented worldwide’ – Ricki Lewis, a science writer with a Ph.D in genetics, wrote in her PLOS article — ‘The Curious Genetics of Werewolves’ that there are only 50 cases known since middle age.