caa a22 4500 465767885 CHVBK 20180323111919.0 cr unu---uuuuu 170327e19900701xx s 000 0 eng 10.1007/BF00294645 doi (NATIONALLICENCE)springer-10.1007/BF00294645 Lectin histochemistry in brains with juvenile form of neuronal ceroid-lipofuscinosis (Batten disease) [Elektronische Daten] [K. Wisniewski, D. Maslinska] Summary: Defective ultilization 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-manose 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 moities in neurons containinglipofuscin 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. Springer-Verlag, 1990 Neuronal ceroid-lipofuscinoses nationallicence Batten disease nationallicence Lipid storage disease nationallicence lectin histochemistry nationallicence Inherited neurodegenerative diseases nationallicence Wisniewski K. NYS Office of Mental Retardation and Developmental Disabilities, Institute for Basic Research, 1050 Forest Hill Road, 10314, Staten Island, NY, USA aut Maslinska D. NYS Office of Mental Retardation and Developmental Disabilities, Institute for Basic Research, 1050 Forest Hill Road, 10314, Staten Island, NY, USA aut Acta Neuropathologica Springer-Verlag 80/3(1990-07-01), 274-279 0001-6322 80:3<274 1990 80 401 https://doi.org/10.1007/BF00294645 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00294645 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Wisniewski K. NYS Office of Mental Retardation and Developmental Disabilities, Institute for Basic Research, 1050 Forest Hill Road, 10314, Staten Island, NY, USA aut NATIONALLICENCE 700 1- Maslinska D. NYS Office of Mental Retardation and Developmental Disabilities, Institute for Basic Research, 1050 Forest Hill Road, 10314, Staten Island, NY, USA aut NATIONALLICENCE 773 0- Acta Neuropathologica Springer-Verlag 80/3(1990-07-01), 274-279 0001-6322 80:3<274 1990 80 401 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer