progeria? what causes it ?? anyone know !!!!?

Answer 1

progeria is a group of conditions charecterized by abnormal aging process .
a 10 year old with this disease would look like and have the problems of a 50 year old.
its mainly caused by certain genes.


Progeria Syndromes
From Mary Kugler,Your Guide to Rare / Orphan Diseases.

Give appearance of premature aging
Many people know progeria from seeing children who look very old on TV talk shows and documentaries. Those children have the more rare form of progeria; there is another more common type that affects adolescents and adults.
Hutchinson-Gilford progeria
This syndrome occurs in about 1 in 8 million children. At birth, a child with Hutchinson-Gilford progeria appears normal. The syndrome begins to show around 6-12 months of age, when the baby fails to gain weight and skin changes occur. Over time, the child begins to look like an elderly person. Some of the characteristic symptoms are:

Head and face: baldness, prominent scalp veins and eyes, small jaw, delayed tooth formation
Bones: thin limbs with prominent joints, short stature, joint stiffness, hip dislocations
Heart disease and artery disease
About 97% of the children affected are Caucasian, but all children with Hutchinson-Gilford progeria have a similar appearance, regardless of racial/ethnic background. Children with the syndrome survive to an average age of 13 years old, with most succumbing to heart disease.

Werner Syndrome
Werner syndrome is a less well-known but more common form of progeria, occurring in about 1 in 1 million individuals. Typically, Werner syndrome is identified when an adolescent fails to have a normal growth spurt. Over time, the young person begins to look elderly. Some of the characteristic symptoms are:

Striking difference between the person's appearance and his/her real age
Head and face: gray hair and/or balding, wrinkling of the face, cataracts, sunken cheeks and small jaw, a high-pitched voice
Bones: osteoporosis, small stature, muscle weakness
Diabetes and cancer common
Werner syndrome appears to occur more often in people of Japanese and Sardinian heritage. People with Werner syndrome survive to an average age of 46 years old, with most succumbing to heart disease or cancer.

Causes
In 1992, researchers pegged Chromosome 8 as being the locus of Werner syndrome; in 2003 the responsible gene was identified. Researchers hope that they will be able to discover what roles this gene plays in the body and thereby figure out the mechanisms behind the syndrome.

The gene responsible for Hutchinson-Gilford syndrome has not been identified, so researchers do not have a grasp yet of what causes the syndrome. They suspect a gene defect (mutation) occurs accidentally in a human embryo, and the child then develops progeria.

May not be premature aging
In both syndromes, research suggests that although the affected person may look like he or she is growing old prematurely, there are a number of conditions that develop that are very different from those of elderly people. For example, people with Werner syndrome develop osteoporosis, as in aging, but their form of the bone disease is unusually severe in the long bones of the legs. Yet researchers are hopeful that both syndromes will give some clues about the genetic factors involved in the aging process.

Answer 2

What Causes Progeria

Answer 3

Progeria narrowly refers to Hutchinson-Gilford Progeria syndrome, but the term is also used more generally to describe any of the so-called "accelerated aging" diseases. The word progeria is derived from the Greek for "prematurely old". Because the "accelerated aging" diseases display different aspects of aging, but never every aspect, they are often called segmental progerias by biogerontologists.

Hutchinson-Gilford Progeria syndrome is an extremely rare genetic condition which causes physical changes that resemble greatly accelerated aging in sufferers. The disease affects between 1 in 4 million (estimated actual) and 1 in 8 million (reported) newborns. Currently, there are approximately 40-45 known cases in the world. There is no known cure. Most people with progeria die around 13 years of age. Progeria is of interest to scientists because the disease may reveal clues about factors involved in the process of aging. Unlike most other "accelerated aging diseases" (such as Werner's syndrome, Cockayne's syndrome or xeroderma pigmentosum), progeria is not caused by defective DNA repair.

The condition was first identified in 1886 by Jonathan Hutchinson and Hastings Gilford. The condition was later named Hutchinson-Gilford Progeria syndrome (HGPS). Around 100 cases have been identified since then.

SYMPTOMS
Symptoms generally begin appearing around 18-24 months of age. The condition is distinguished by limited growth, alopecia and a characteristic appearance with small face and jaw and pinched nose. Later the condition causes wrinkled skin, atherosclerosis and cardiovascular problems. Mental development is not affected. Individuals with the condition rarely live more than 16 years; the longest recorded life-span was 29 years. The development of symptoms is comparable to aging at a rate six to eight times faster than normal, although certain age-related conditions do not occur. Specifically, victims show no neurodegeneration or cancer predisposition

CURE?
A cure for progeria has not yet been found.

Answer 4

Hutchinson-Gilford Progeria Syndrome ("Progeria", or "HGPS") is a rare, fatal genetic condition. It is not hereditary.
It is caused by a mutation in the gene called LMNA. This gene produces a protein that basically holds cells together, without it, the cells become unstable which leads to a process of premature aging.
Children with progeria usually die of heart disease in their early teens. Another form of the disease can start in the teen years, with an expected lifespan into the 40's to 50's.

Answer 5

"Progeria is a rare condition that is remarkable because its symptoms strongly resemble normal human aging, but occur in young children. In addition, the appearance of several affected children in movies and on television have brought progeria to public attention. Ninety percent of children with progeria have a mutation on the gene that encodes the protein lamin A. Progeria usually occurs sporadically, and is not seen in siblings of affected children, although -- in very rare circumstances -- more than one child in a family can have progeria."

Answer 6

Out of 25, 000 dna pairs that make up the LMNA gene, 1 of those pairs is missing , this gene, stabilizes the inner membrane of the cell's nucleus

Ps. I didn't just cut in paste, I chose the seemingly most important part of what causes it and rephrased it, in a way that I could understand a little better, and probably everyone else can as well

Answer 7

Progeria is an extremely rare genetic disease of childhood characterized by dramatic, premature aging. The condition, which derives its name from "geras," the Greek word for old age, is estimated to affect one in 8 million newborns worldwide.

The most severe form of the disease is Hutchinson-Gilford progeria syndrome, recognizing the efforts of Dr. Jonathan Hutchinson, who first described the disease in 1886, and Dr. Hastings Gilford who did the same in 1904.

As newborns, children with progeria usually appear normal. However, within a year, their growth rate slows and they soon are much shorter and weigh much less than others their age. While possessing normal intelligence, affected children develop a distinctive appearance characterized by baldness, aged-looking skin, a pinched nose, and a small face and jaw relative to head size. They also often suffer from symptoms typically seen in much older people: stiffness of joints, hip dislocations and severe, progressive cardiovascular disease. However, various other features associated with the normal aging process, such as cataracts and osteoarthritis, are not seen in children with progeria.

Some children with progeria have undergone coronary artery bypass surgery and/or angioplasty in attempts to ease the life-threatening cardiovascular complications caused by progressive atherosclerosis. However, there currently is no treatment or cure for the underlying condition. Death occurs on average at age 13, usually from heart attack or stroke.

In 2003, NHGRI researchers, together with colleagues at the Progeria Research Foundation, the New York State Institute for Basic Research in Developmental Disabilities, and the University of Michigan, discovered that Hutchinson-Gilford progeria is caused by a tiny, point mutation in a single gene, known as lamin A (LMNA). Parents and siblings of children with progeria are virtually never affected by the disease. In accordance with this clinical observation, the genetic mutation appears in nearly all instances to occur in the sperm prior to conception. It is remarkable that nearly all cases are found to arise from the substitution of just one base pair among the approximately 25,000 DNA base pairs that make up the LMNA gene.

The LMNA gene codes for two proteins, lamin A and lamin C, that are known to play a key role in stabilizing the inner membrane of the cell's nucleus. In laboratory tests involving cells taken from progeria patients, researchers have found that the mutation responsible for Hutchinson-Gilford progeria causes the LMNA gene to produce an abnormal form of the lamin A protein. That abnormal protein appears to destabilize the cell's nuclear membrane in a way that may be particularly harmful to tissues routinely subjected to intense physical force, such as the cardiovascular and musculoskeletal systems.

Interestingly, different mutations in the same LMNA gene have been shown to be responsible for at least a half-dozen other genetic disorders, including two rare forms of muscular dystrophy.

In addition to its implications for diagnosis and possible treatment of progeria, the discovery of the underlying genetics of this model of premature aging may help to shed new light on humans' normal aging process.

there is no cure

Answer 8

It is due to something going wrong with a gene, during development- it is not hereditary though. You can read more about it here
http://www.progeriaresearch.org/progeria_101.html

Answer 9

Never heard of it. Look it up at WebMD