Canavan disease is a progressive, fatal neurological disorder that begins in infancy. It is caused by an inherited genetic abnormality: the lack of an essential enzyme causes deterioration of the white matter (myelin) in the brain, thereby preventing the proper transmission of nerve signals.
Canavan disease is an inherited disorder that destroys the myelin sheath, the white matter that insulates nerve cells in the brain. It causes overall muscle weakness and developmental delay leading to severe mental disability. Canavan disease is caused by a deficiency in an enzyme called aspartoacylase. This enzyme breaks down a material called N-acetyl-L-aspartic acid (NAA) in the brain. Without enough enzyme, the NAA builds up in the brain and destroys its white matter. Canavan disease is a rare inherited disorder that damages the ability of nerve cells (neurons) in the brain to send and receive messages. This disease is one of a group of genetic disorders called leukodystrophies. Leukodystrophies disrupt the growth or maintenance of the myelin sheath, which is the covering that protects nerves and promotes the efficient transmission of nerve impulses. Canavan disease is a progressive, fatal neurological disorder that begins in infancy. It is caused by an inherited genetic abnormality: the lack of an essential enzyme causes deterioration of the white matter (myelin) in the brain, thereby preventing the proper transmission of nerve signals.  From Wikipedia, the free encyclopedia. Canavan disease, also called Canavan-Van Bogaert-Bertrand disease is an autosomal recessive degenerative disorder that causes progressive damage to nerve cells in the brain, and is one of the most common degenerative cerebral diseases of infancy. Canavan disease has an autosomal recessive pattern of inheritance. Canavan disease is inherited in an autosomal recessive fashion. When both parents are carriers, there is a 25% chance of having an affected child. Canavan disease has been classified as one of a group of genetic disorders known as the leukodystrophies. Recent research has indicated that the cells in the brain responsible for making myelin sheaths, known as oligodendrocytes, cannot properly complete this critical developmental task. Canavan disease is caused by mutation in the gene for an enzyme called aspartoacylase, which acts to break down the concentrated brain chemical known as N-acetyl-aspartate. Canavan disease (CD) belongs to a group of conditions known as leukodystrophies, characterized by defects in myelin, commonly known as the white matter in the brain. Myelin protects nerves and allows messages to be sent to and from the brain. All CD symptoms are explained by the progressive loss of myelin.  Canavan disease is a gene-linked neurological disorder in which the brain degenerates into spongy tissue riddled with microscopic fluid-filled spaces. Canavan disease is caused by mutation in the gene for an enzyme called aspartoacylase, which acts to break down the concentrated brain chemical known as N-acetyl-aspartate. Canavan disease. Canavan disease is a leukodystrophy-one of several devastating neurological disorders in which the brain deteriorates due to a defective inherited gene. Like many other identified leukodystrophies, Canavan Disease interferes with the body's normal production of myelin. This fatty membrane, the central nervous system's white matter, forms a protective coating around each nerve in the brain and spinal cord, ensuring that nerve impulses are properly transmitted. 1 Canavan disease is a gene-linked neurological disorder that results in brain tissue degeneration. 2  The cause of the disease is a genetic disorder that causes oligodendrocytes to remain immature and then die, thereby not completing the synthesis of myelin sheaths. *Canavan disease facts medical author: Charles Patrick Davis, MD, PhD. 1 
https://www.counsyl.com/diseases/canavan-disease/ http://ghr.nlm.nih.gov/condition/canavan-disease http://www.canavanfoundation.org/about_canavan_disease https://en.wikipedia.org/wiki/Canavan_disease http://www.ninds.nih.gov/disorders/canavan/canavan.htm http://www.tay-sachs.org/canavan_disease.php http://www.ninds.nih.gov/disorders/canavan/canavan.htm http://canavan.org/canavan-disease.html http://www.medicinenet.com/canavan_disease/article.htm
what is canavan disease