Premium
Renal cerebroside in globoid cell leukodystrophy (Krabbées disease)
Author(s) -
Suzuki Kunihiko
Publication year - 1971
Publication title -
lipids
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.601
H-Index - 120
eISSN - 1558-9307
pISSN - 0024-4201
DOI - 10.1007/bf02531383
Subject(s) - galactocerebroside , glucocerebroside , cerebroside , leukodystrophy , krabbe disease , kidney , gaucher's disease , biology , pathology , glucocerebrosidase , medicine , enzyme , biochemistry , disease , myelin , endocrinology , central nervous system , oligodendrocyte
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.