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Acta Neuropathologica

, Volume 76, Issue 4, pp 359–365 | Cite as

Lectin histochemistry of gangliosidosis

II. Neurovisceral tissues from patients with Sandhoff's disease
  • J. Alroy
  • L. S. Adelman
  • C. D. Warren
Regular Papers

Summary

Lectin histochemical studies were performed on selected formalin-fixed, paraffin-embedded tissues of patients affected with the O variant of GM2-gangliosidosis (i.e., Sandhoff's disease). The purpose was to identify specific sugar residues of undegraded “stored” substances in cytoplasm of affected cells. We studied neural tissues from 13 patients, visceral tissues from four patients, and placentae from three affected fetuses. Neurons in all 13 cases studied stained withConcanavalia ensiformis agglutinin (Con A) and withUlex europaeus agglutinin-I (UEA-I). Succinylated wheat germ agglutinin (S-WGA) stained affected visceral cells and astrocytes and macrophages in the central nervous system. These results demonstrate that α-d-mannosyl and α-l-fucosyl residues, which bind Con A and UEA-I, respectively, are present in affected neurons. Furthermore, they revealed the affected nonneuronal cells and astrocytes contain complex carbohydrates with nonreducing terminal β-N-acetylglucosamine, which binds S-WGA.

Key words

Hexosaminidase A and B deficiency Lectin histochemistry 

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Copyright information

© Springer-Verlag 1988

Authors and Affiliations

  • J. Alroy
    • 1
  • L. S. Adelman
    • 1
    • 2
  • C. D. Warren
    • 3
  1. 1.Department of PathologyTufts University Schools of Medicine and Veterinary Medicine and New England Medical CenterBostonUSA
  2. 2.Department of NeurologyTufts University Schools of Medicine and Veterinary Medicine and New England Medical CenterBostonUSA
  3. 3.Laboratory for Carbohydrate Research, Department of Biological Chemistry and MedicineHarvard Medical School and Massachusetts General HospitalBostonUSA

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