What is a glial fibrillar acid protein?

Glioine fibrillary acidic protein (GFAP) is one of the group of proteins that make up building blocks of what is called mean fibers. Middle fibers are found in astroglial cells that help keep the brain and spinal cord. Astroglial cells are expected to play an important role in myelin formation, allowing impulses to pass through nerves, and help create a brain blood barrier that controls which substances move from circulation to the brain. When genes are defective for glial fibrillary acid protein, GFAP has an abnormal structure. This can cause what is called Alexander's disease, which is a rare condition in which brain tissue is gradually destroyed. The Strand Protein Springs of the Middle Fiber are joined to form networks that are part of the skeleton of the astroglial cell, allowing it to unite and help it maintain shape. It is believed that astroglial cells affect the cells surrounding the nerves, allowing them to produce a type of fat called myelin that forms an insulating coat.

When a genetic disorder causes mutation in the genk of glial fibrillary acid protein, this can lead to the production of abnormal GFAP. It is assumed that defective glial fibrillary acid protein prevents the normal process of forming intermediates and abnormal gfap accumulates in cells. In Alexander's disease in astroglial cells, collections of protein known as Rosenthal fibers appear. It is assumed that glial fibrillary acid protein contributes to this formation of fibers. Astroglial cells no longer work properly and Myelin becomes abnormal.

In Alexander's disease, the loss of myelin means that nerve impulses are not transmitted correctly. The condition may develop in infancy, childhood or maturity. What is called an infantile form is the most common, with symptoms and symptoms observed in the first two years of life. Brain and head, limb stiffness, seizures and intellectual damage may increase.The inability of youthful and adults Alexander is associated with seizures, poor coordination and difficulty in speech and swallowing.

While most patients with Alexander have a mutation in the gli fibrillary acid protein gene, the disorder is not always inherited. Although the condition is fatal and there is no medicine, the view for people with Alexanders' disease is quite variable. The infantile type of disease is usually fatal under six years, but patients with other forms usually survive longer, sometimes for decades.