Progeria?
- Literature - Therapy for Progeria
Therapy for Progeria
Biol. 2402 - Anatomy & Physiology
Richland College, Spring 2001 Class Project
Web Project Members:
Amanda Williams, Tia Furcht, Kristin Kosarek
It is important to note that there is no proven therapy as a cure for Progeria; however, there are theories that are based on clinical research that hope to further develop into possible cures…
Since Progeria patients have commonly low levels of antioxidants in the body, there are alternative therapies to combat oxidative stress with: [www.anti-aging.com]
Immediate Solutions:
- Prescribed formulas of antioxidants including vitamins, coenzyme Q-10, and
lipoic acid
- Vitamin E for its antioxidant effects
- Treatment of Progeria with growth hormone
What is the Destruction of
Cells by Oxidative Stress?
Oxygen levels are drastically impaired by high amounts of free radicals. Natural
metabolic processes, toxins in the environment, and disease contribute to
increased damage by radicals. Under normal conditions, enzymes called
antioxidants break down free radicals and prevent the damage of body lipids,
protein, and DNA. When free radicals supercede the amount of antioxidants in the
body, the result is oxidative stress. Superoxide dismutase and catalase enzymes
convert free radicals into benign substances such as water and oxygen. However,
when these enzymes are found in small mounts, this can deplete the motor neurons
of the oxygen they need to function. They are significant factors in determining
the onset of the process of aging.
The nervous system is the most vulnerable system due to the amount of oxygen it consumes.
Long term solutions: Genetic engineering can now be used to deliver genes that code for the enzymes into Progeria patients.
Enzyme percentages: Progeria sufferers have only half of the normal caltase production of a normal metabolic percentage and only 30% of the glutathionine production required. Restoring their 80% deficient enzyme levels to normal could be the first step to combating the cause of this disease. If the right enzymes are produced it will halt the hyperdestruction of cells.
“Radical Medical Advancement” - The University of Washington is currently reintroducing enzymes that may halt or reverse the problem of age all together by using adenovirus gene transfers. By attaching enzymes to the common cold virus, the transporter (the cold virus) takes enzymes directly into the cell. [http://www.genetics.com]
Telomeres Theory
“The shortening of telomeres”
Once the telomeres reach a certain length on the chromosome, they cease to
reproduce and the process of aging begins. A Progeria patient ages at a rate of
seven times the normal aging process; therefore, targeting the area of actual
rapid growth rate is of important consideration. Genetically defective telomeres
could allow for cells to age faster and die faster than in a normal person.
Telomeres are important because they protect chromosomes from degradation and
fusion.
Since cellular aging is a genetically determined process, telomeres are the molecular clock for cellular aging. Manipulating the length of telomeres alters the life span of human cells.
When the gene that codes for the enzyme telomerase functions incorrectly they an build up at the end of genes and when the genes become shorter, they will stop dividing.
Human teleomerase composition - It is commonly found in immortal cells such as sperm. The catalytic component is hTERT enzyme.
New scientific findings by http://www.geron.com believe that telomeres can now be cloned and then made into a drug that encourages cells to make their own telomerase.
Dr. Calvin Harley - Dr. Harley has made dramatic advances in gene therapy by injecting the telomerase gene directly into eye cells. He believes that with progress and medical advancement of these studies, a safe delivery and activation of telomerase can help progeria children with shortened telomeres.
Copyright © The Progeria Project Foundation