Gangliosides in Human Skin Fibroblasts and their Enrichment in the “Hurler Variant” and Krabbe's Disease

Liselotte Hof, Reuben Matalon, Albert Dorfman

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

Structure and quantities of gangliosides in skin fibroblasts derived from normal children and patients with several hereditary diseases are studied. Mono and disialohematosides are the only gangliosides found. There was a 6-fold increase in lipid-bound neuraminic acid in the Hurler variant and 2.3-fold increase in Krabbe's diesease as compared to the highest normal cells grown under the same conditions. The addition of calf-serum to the growth medium caused a substantial increase in the hematosides and also the occurrence of N-glycoloylneuraminic acid besides N-acetylneuraminic acid.

Original languageEnglish (US)
Pages (from-to)1329-1337
Number of pages9
JournalHoppe-Seyler's Zeitschrift fur Physiologische Chemie
Volume352
Issue number2
DOIs
StatePublished - 1971
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry

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