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What is Krabbe Leukodystrophy?

How do you pronounce Krabbe Disease? What causes Krabbe? Why doesn’t Krabbe show up at birth? How common is Krabbe Leukodystrophy? Become aware…

What is Krabbe Disease? Krabbe disease (pronounced Krahb-A), also known as Globoid Cell Leukodystrophy, is a rare, genetic, currently-incurable and ultimately fatal disorder. Krabbe Disease is one of several known leukodystrophies: genetic diseases that progressively destroy the white matter of the brain.  In Krabbe disease, the enzyme galactocerebrosidase (GALC), which is essential for metabolizing several important compounds in the body, is deficient. Therefore, psychosine, a toxic substance which should be broken down and naturally released from the body starts to accumulate. This toxic build-up damages the central nervous system, particularly destroying the myelin sheath, which insulates the nerves. The destruction of the myelin sheath, called demyelination, impedes the conduction of nerve signals from the brain to the rest of the body. As the myelin degrades, it causes severe physical disabilities.

 

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KRABBE DISEASE

Krabbe (Krahb-A) disease, also known as Globoid Cell Leukodystrophy, is a rare, genetic, currently-incurable and ultimately fatal disorder. It is one of several known leukodystrophies: genetic diseases that progressively destroy the white matter of the brain.

In Krabbe disease, the enzyme galactocerebrosidase (GALC), which is essential for metabolizing several important compounds in the body, is deficient. Therefore, psychosine, a toxic substance which should be broken down and naturally released from the body starts to accumulate. This toxic build-up damages the central nervous system, particularly destroying the myelin sheath, which insulates the nerves. The destruction of the myelin sheath, called demyelination, impedes the conduction of nerve signals from the brain to the rest of the body. As the myelin degrades, it causes severe physical disabilities.

 

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