Studies of a synthetic substrate in canine globoid cell leukodystrophy

Thaddeus W. Kurczynski, Sophia K. Kondoleon, Robert G. Macbride, Lois H. Dickerman, Thomas F. Fletcher

Research output: Contribution to journalArticlepeer-review

1 Scopus citations


Gal et al. ((1977) Clin. Chim. Acta 77, 53-59) reported the use of a new synthetic substrate, 2-hexadecanoylamino-4-nitrophenyl-β-D-galactopyranoside for the diagnosis of human globoid cell leukodystrophy. Assay of β-galactosidase in brain homogenates from normal, carrier, and globoid cell leukodystrophy-affected dogs utilizing this new substrate demonstrated overlapping activities. Instead of reflecting specific D-galactosyl-N-acylsphingosine galactohydrolase (EC, the 2-hexadecanoylamino-4-nitrophenyl-β-D-galactopyranoside β-galactosidase activity in canine brain is highly correlated with nonspecific 4-methylumbelliferyl β-galactosidase. Optimization of the 2-hexadecanoylamino-4-nitrophenyl-β-D-galactopyranoside assay system for canine brain and the use of varying concentrations of taurocholate or taurodeoxycholate in the assay mixture did not alter the lack of specificity. These results indicate a significant difference in the nature of the underlying defect in galactosylceramide β-galactosidase in canine globoid cell leukodystrophy compared to human globoid cell leukodystrophy.

Original languageEnglish (US)
Pages (from-to)297-302
Number of pages6
JournalBBA - General Subjects
Issue number3
StatePublished - Feb 5 1981


  • (Canine globoid cell)
  • Leukodystrophy
  • Synthetic substrate
  • β-Galactosidase


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