Galactocerebrosidase - Volpe 1 Rome Volpe Professor Cadogan...

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Volpe 1 Rome Volpe Professor Cadogan Biology Lab 16 Nov. 2017 Galactocerebrosidase: The Krabbe Enzyme Galactocerebrosidase, or GALC, is a lysosomal enzyme that hydrolyzes, or uses water molecules to break down, specific fats known as galactolipids. These galactolipids are a form of glycolipid whose particular sugar group is galactose. These lipids are extremely important and can be found in kidney, small intestine, and myelin cells. There are numerous different galactolipids, however, the accrual of psychosine seems to be particularly harmful and directly responsible for major complications. Galactocerebrosidase is found primarily in the brain and kidneys. A deficiency of Galactocerebrosidase will likely lead to an autosomal recessive neurodegenerative disorder known as globoid cell leukodystrophy (GLD) or Krabbe disease. This GALC deficiency directly leads to an amassing of cytoxic metabolite, which causes the apoptosis of myelin-forming cells to transpire (Visigalli). This disease affects the central nervous system, and the peripheral nervous system, due to demyelination. Demyelination arises when the nerves insulating coat; the myelin sheath, which is comprised of a massive network of neurons,

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