Studies of a synthetic substrate in canine globoid cell leukodystrophy.

Abstract

Gal et al. ((1977) Clin. Chim. Acta 77, 53-59) reported the use of a new synthetic substrate, 2-hexadecanoylamino-4-nitrophenyl-beta-D-galactopyranoside for the diagnosis of human globoid cell leukodystrophy. Assay of beta-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 3.2.1.46), the 2-hexadecanoylamino-4-nitrophenyl-beta-D-galactopyranoside beta-galactosidase activity in canine brain is highly correlated with nonspecific 4-methylumbelliferyl beta-galactosidase. Optimization of the 2-hexadecanoyl-amino-4-nitrophenyl-beta-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 beta-galactosidase in canine globoid cell leukodystrophy compared to human globoid cell leukodystrophy.

• Authors: T W Kurczynski, S K Kondoleon, R G MacBride, L H Dickerman, T F Fletcher
• Journal: Biochimica et biophysica acta (Biochim Biophys Acta) Vol. 672 Issue 3 Pg. 297-302 (Feb 05 1981) ISSN: 0006-3002 [Print] Netherlands
• PMID: 6783123 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Galactosides
• Glycosides
• Nitrophenols
• 2-hexadecanoylamino-4-nitrophenyl-beta-D-galactopyranoside
• Galactosidases
• 4-methylumbelliferyl-beta-galactosidase
• beta-Galactosidase

Topics

• Animals
• Brain (enzymology)
• Dogs
• Galactosidases (analysis)
• Galactosides
• Glycosides
• Leukodystrophy, Globoid Cell (diagnosis, enzymology)
• Nitrophenols
• beta-Galactosidase (analysis)