Lectin histochemistry in brains with juvenile form of neuronal ceroid-lipofuscinosis (Batten disease).
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.
NYS Office of Mental Retardation and Developmental Disabilities, Institute for Basic Research, Staten Island, NY 10314.
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Wisniewski
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Maslinska
D000293:Adolescent; D000328:Adult; D000368:Aged; D000375:Aging / Q000378:metabolism; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D002648:Child; D006651:Histocytochemistry; D006801:Humans; D037102:Lectins; D008062:Lipofuscin / Q000378:metabolism; D009472:Neuronal Ceroid-Lipofuscinoses / Q000378:metabolism* / Q000473:pathology; D009474:Neurons / Q000378:metabolism
D037102:Lectins; D008062:Lipofuscin