aragonite; aragonitic; AraguaÃ­a; Araguaia River; Araguato; Araguaya; Araguaya River; arain; araise; arak; Arakan Yoma; Araks; Aral Sea; Araldite; Arales; aralia; Aralia elata; Aralia hispida; Aralia nudicaulis; Aralia racemosa; Aralia spinosa; Aralia stipulata; Araliaceae; araliaceous; Aram; Aram Ilich Khachaturian; Aram Kachaturian
Krabbe disease (also known as globoid cell leukodystrophy or galactosylceramide lipidosis) is a rare, often fatal degenerative disorder that affects the myelin sheath of the nervous system.It is a form of sphingolipidosis, as it involves dysfunctional metabolism of sphingolipids.he disease is named after the Danish neurologist Knud Haraldsen Krabbe (1885-1965). Illinois, Kentucky and Pennsylvania have laws to test for Krabbe and other diseases at birth. There is a movement in Maine and other states to adopt such regulations.
Each of the leukodystrophies affects one (and only one) of these substances. Krabbe disease is caused by a deficiency of galactocerebrosidase, an essential enzyme for myelin metabolism.The disease most often affects infants, with onset before age 6 months, but can occur in adolescence or adulthood.t is characterized by the presence of globoid cells (cells that have more than one nucleus), the breakdown of the nerveâs protective myelin coating, and destruction of brain cells. Krabbe disease is one of a group of genetic disorders called the leukodystrophies.