Clinical, electrophysiological, and biochemical markers of peripheral and central nervous system disease in canine globoid cell leukodystrophy (Krabbe's disease).

@article{Bradbury2016ClinicalEA,
  title={Clinical, electrophysiological, and biochemical markers of peripheral and central nervous system disease in canine globoid cell leukodystrophy (Krabbe's disease).},
  author={Allison M. Bradbury and Jessica H. Bagel and Xuntian Jiang and Gary P. Swain and Maria L Prociuk and Caitlin A. Fitzgerald and Patricia A. O'Donnell and Kyle G Braund and Daniel S Ory and Charles Herman Vite},
  journal={Journal of neuroscience research},
  year={2016},
  volume={94 11},
  pages={
          1007-17
        }
}
Globoid cell leukodystrophy (GLD), or Krabbe's disease, is a debilitating and always fatal pediatric neurodegenerative disease caused by a mutation in the gene encoding the hydrolytic enzyme galactosylceramidase (GALC). In the absence of GALC, progressive loss of myelin and accumulation of a neurotoxic substrate lead to incapacitating loss of motor and cognitive function and death, typically by 2 years of age. Currently, there is no cure. Recent convincing evidence of the therapeutic potential… CONTINUE READING
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