Progeria
Progeria
Progeria, also known as Hutchinson-Gilford Progeria syndrome (HGPS), is a rare disease that causes children to age eight times faster than they are supposed to. Progeria was named after Jonathan Hutchinson who first discovered it while living in England, and Hastings Gilford, who was the first to refer to the disease as Progeria in 1886. The name Progeria is derived from Greek and means "prematurely old". Progeria is commonly referred to as an "early aging disease,” and fortunately an extremely rare disease. Only 100 cases have been reported since HGPS was discovered; It is estimated to affect only one to two children in eight million. There are currently about forty children around the world that are affected with the disease. It is, however, a particularly tragic disease in that it typically strikes children before the age of ten years; They usually die at the age of thirteen. Even though most children don't live beyond their teenage years, there are one or two children affected with Progeria that have lived to be in their early twenties. The life-span for children with Progeria ranges from seven to twenty seven and half years old. (Progeria,1)
The Science behind Progeria
Hutchinson – Gilford Progeria Syndrome (HGPS) is caused by a mutation in the gene called LMNA, pronounced Lamin. The LMNA gene produces a Lamin A protein. The job of this protein is to hold the nucleus of the cell together. Researchers believe that the defective Lamin A protein makes the nucleus unstable, thus leading to the process of premature aging in Progeria.(Reversal of the cellular phenotype, 1) The children with Progeria are born looking healthy, then begin to display many characteristics of accelerated aging around 18-24 months. HGPS signs include but not limited to, growth failure, loss of body fat and hair, aged looking skin, stiffness of joints, hip dislocation, hardening of arteries, heart disease and stroke. All children diagnosed with Progeria all have a remarkable
Progeria, also known as Hutchinson-Gilford Progeria syndrome (HGPS), is a rare disease that causes children to age eight times faster than they are supposed to. Progeria was named after Jonathan Hutchinson who first discovered it while living in England, and Hastings Gilford, who was the first to refer to the disease as Progeria in 1886. The name Progeria is derived from Greek and means "prematurely old". Progeria is commonly referred to as an "early aging disease,” and fortunately an extremely rare disease. Only 100 cases have been reported since HGPS was discovered; It is estimated to affect only one to two children in eight million. There are currently about forty children around the world that are affected with the disease. It is, however, a particularly tragic disease in that it typically strikes children before the age of ten years; They usually die at the age of thirteen. Even though most children don't live beyond their teenage years, there are one or two children affected with Progeria that have lived to be in their early twenties. The life-span for children with Progeria ranges from seven to twenty seven and half years old. (Progeria,1)
The Science behind Progeria
Hutchinson – Gilford Progeria Syndrome (HGPS) is caused by a mutation in the gene called LMNA, pronounced Lamin. The LMNA gene produces a Lamin A protein. The job of this protein is to hold the nucleus of the cell together. Researchers believe that the defective Lamin A protein makes the nucleus unstable, thus leading to the process of premature aging in Progeria.(Reversal of the cellular phenotype, 1) The children with Progeria are born looking healthy, then begin to display many characteristics of accelerated aging around 18-24 months. HGPS signs include but not limited to, growth failure, loss of body fat and hair, aged looking skin, stiffness of joints, hip dislocation, hardening of arteries, heart disease and stroke. All children diagnosed with Progeria all have a remarkable