Renal cerebroside in globoid cell leukodystrophy (Krabbées disease) | Zendy

Suzuki Kunihiko | Zendy

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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.

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