Krabbe disease (KD) is an autosomal recessive sphingolipidosis caused by the deficiency\nof the lysosomal hydrolase Beta-galactosylceramidase (GALC). Oligodendroglia degeneration and\ndemyelination of the nervous system lead to neurological dysfunctions which are usually lethal by\ntwo years of age. At present, the only clinical treatment with any proven efficacy is hematopoietic\nstem-cell transplantation, which is more effective when administered in the neonatal period to\npresymptomatic recipients. Bone marrow (BM) sinusoidal endothelial cells (SECs) play a pivotal\nrole in stem cell engraftment and reconstitution of hematopoiesis. Previous observations had shown\nsignificant alterations of microvascular endothelial cells in the brain of KD patients and in.............
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