Galactocerebrosidase-deficient oligodendrocytes maintain stable central myelin by exogenous replacement of the missing enzyme in mice.

@article{Kondo2005GalactocerebrosidasedeficientOM,
  title={Galactocerebrosidase-deficient oligodendrocytes maintain stable central myelin by exogenous replacement of the missing enzyme in mice.},
  author={Yoichi Kondo and Deborah A. Wenger and V. de Miguel Gallo and Ian D. Duncan},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={2005},
  volume={102 51},
  pages={
          18670-5
        }
}
Globoid cell leukodystrophy (GLD) is a lysosomal storage disease caused by genetic deficiency of galactocerebrosidase (GALC) activity. Failure in catalyzing the degradation of its major substrate, galactocerebroside, in oligodendrocytes (OLs) and Schwann cells leads to death of these myelinating cells, progressive demyelination, and early demise of GLD patients. Transplantation of bone marrow cells and umbilical cord blood have been attempted as a means of enzyme replacement and have shown… CONTINUE READING

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