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.
Wisniewski
K E
KE
NYS Office of Mental Retardation and Developmental Disabilities, Institute for Basic Research, Staten Island, NY 10314.
Maslinska
D
D
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