Immuno-lectin histochemistry and ultrastructure in two cases of globoid cell leukodystrophy (Krabbe's disease)

Clin Neuropathol. 1992 Nov-Dec;11(6):312-7.

Abstract

Two cases of globoid cell leukodystrophy (Krabbe's disease) have been studied by means of conventional histological, immunohistochemical and ultrastructural methods. Lectins specific for several different carbohydrates, Ricinus communis (RCA-1), peanut agglutinin (PNA), wheat germ agglutinin (WGA) and Concanavalin A (Con A) have been utilized both at optical and ultrastructural level. In both cases a strong positive labeling of the globoid cells could be observed light-microscopically. Ultrastructurally immunogold deposits only of RCA-I and WGA in the typical Krabbe's inclusions have been clearly demonstrated.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acetylgalactosamine / analysis
  • Acetylglucosamine / analysis
  • Blood Glucose / analysis
  • Brain / pathology*
  • Cytoplasm / ultrastructure
  • Galactose / analysis
  • Humans
  • Immunoenzyme Techniques
  • Inclusion Bodies / ultrastructure
  • Infant
  • Leukodystrophy, Globoid Cell / genetics
  • Leukodystrophy, Globoid Cell / pathology*
  • Male
  • Mannose / analysis
  • Microscopy, Electron
  • Myelin Sheath / pathology
  • N-Acetylneuraminic Acid
  • Neurons / pathology
  • Receptors, Mitogen / analysis*
  • Sialic Acids / analysis

Substances

  • Blood Glucose
  • Receptors, Mitogen
  • Sialic Acids
  • N-Acetylneuraminic Acid
  • Acetylgalactosamine
  • Mannose
  • Acetylglucosamine
  • Galactose