Lectin Histochemistry in Brains with Juvenile Form of Neuronal Ceroid-lipofuscinosis (Batten Disease)

Overview

Journal Acta Neuropathol

Specialty Neurology

Date 1990 Jan 1

PMID 2399809

Citations 4

Authors

K E Wisniewski

D Maslinska

Affiliations

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Abstract

Defective utilization of dolichols in the synthesis of glycoprotein leads to an accumulation of the storage, pigment "ceroid" lipofuscin, containing high-mannose-type glycoconjugates, in brains affected by neuronal ceroid-lipofuscinoses (NCL). We have employed lectin histochemistry to study the distribution of such compounds and the composition of other glycoconjugates in brains of patients with a juvenile form of the disease (JNCL). Concanavalin A detected the high-mannose glycoconjugates in all neurons of brains with JNCL, in lipofuscin-containing neurons of aging brains and in some neurons of age-matched control brains. Three other lectins (soybean agglutinin, Peanut agglutinin and Ulex europaeus agglutinin-I) recognized sugar moieties in neurons containing lipofuscin in patients only with JNCL and not in age-matched or aging brains. The results led to the conclusion, that the binding pattern of these three lectins may differentiate between storage materials of NCL and aging brains.

Citing Articles

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Katz M, Buckley R, Biegen V, OBrien D, Johnson G, Warren W G3 (Bethesda). 2020; 10(8):2741-2751.

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Neuronal ceroid lipofuscinoses: a review.

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Identification of lectin binding sites in the rat brain.

Zambenedetti P, Giordano R, Zatta P Glycoconj J. 1996; 13(3):341-6.

PMID: 8781963 DOI: 10.1007/BF00731465.

Lectin histochemistry of lipofuscin and certain ceroid pigments.

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