Renal cerebroside in globoid cell leukodystrophy (Krabbées disease)

Abstract The kidney tissues of five patients with globoid cell leukodystrophy (Krabbées disease) and of seven agematched normal individuals were analyzed for the content of galacto‐and glucocerebrosides. There was a statistically significant increase of galactocerebroside (+25%) in the pathological specimens. However, glucocerebroside was also similarly increased (+30%). Therefore, despite the genetic defect of the degradative enzyme, galactocerebroside β‐galactosidase, there is no specific accumulation of galactocerebroside in the kidneys of patients with globoid cell leukodystrophy.